CHARGE syndrome (CS) is known for causing a wide variety of developmental anomalies. There are many reports that have dealt with CS extensively (Sánchez et al. 2019; van Ravenswaaij-Arts and Martin 2017; de Geus et al., 2017; Hudson et al. 2017; Blake et al. 1998). In 1998, a group of experts defined major and minor criteria of CS (Blake et al., 1998). Accordingly, major criteria or 4Cs include coloboma, choanal atresia, cranial nerve anomalies, and characteristic ear anomalies. Minor criteria are cardiovascular malformations, genital hypoplasia, cleft lip/palate, and tracheoesophageal fistula. Additionally, there is a third group of criteria, occasional findings, that are more prevalent than originally predicted (Issekutz et al., 2005; Schussler et al. 2021). Occasional findings involve musculoskeletal anomalies and malformation. The case we presented showed cardiac anomalies, gastrointestinal malrotation, visceral herniation, and unilateral kidney anomaly. Considering the plethora of clinical case report and series, the presented case will be discussed from developmental perspective. Cardiac development is a complex dynamic process which begins at the 4th week. Dynamics of the developing heart, in particular compartmentalization of cardiac cavity and vascular septation, are not merely governed by cardiac tissue cellular factors, but there are extrinsic factors migrating to developing heart such as neural crest cells (Morton et al. 2017). Additionally, recent findings have provided compelling evidence that indicate the link between the neurodevelopment with congenital heart diseases (Webb et al. 2003). In this case, atrial septal defect (ASD), displaced aortic orifice, and persisted aortico-pulmonary trunk were noticed. Although the mechanism underlying cardiac outflow septation remains controversial, the structure often referred to as the aorto-pulmonary (AP) septum has been suggested to play key roles during separation of the initially single outflow vessel to form the ascending aorta and the pulmonary trunk (Henderson et al. 2020; Bradshaw et al. 2009). Study by Bradshaw et al. (2009) showed that neural crest cell contributes actively to cardiac out flow development. Neeb et al. (2013) classified cardiac out flow tract (OFT) anomalies into four major types. These anomalies include overriding aorta (OA), transposition of great arteries (TGA), double outlet right ventricle (DORV), and persistent truncus arteriosus (PTA). Accordingly, the presented case can be classified as a mixed type of DROV and PTA. Besides the OFT anomalies, ASD should also be added to these anomalies. To the best of our knowledge, this variant of cardiac anomalies has not been reported by previous studies. Another group of findings were severe gastrointestinal abnormalities. Herniated intestinal lopes in the lesser sac, right side descending colon, duodenal pouch, absence of pancreas, and left cystic kidney were the major findings. The parts of the large intestine derive from the midgut and hindgut. By 10 weeks, the abdomen has developmentally enlarged sufficiently so that the entire midgut can be accommodated inside it. Following a further 180° counterclockwise rotation around the superior mesenteric artery, the small intestine returns to the abdominal cavity. Concurrently, the large intestine follows its rotation and also moves 180° counterclockwise. Following the return of midgut in the abdomen, the mesenteries of cecum and ascending colon fix to the dorsal wall, making these parts immobile (Park et al. 2005; Kluth et al. 2003; Kostouros et al. 2020). Although the molecular and structural interactions involved in intestinal rotation that promote the phenomenon of rotation have not been investigated in detail, there is evidence to indicate that the dorsal mesentery of the midgut loop presents molecular and architectural left-right asymmetry. For instance, the transcriptional factor Pitx-2is restrictedly expressed in the left side of the dorsal mesentery in its whole dorsal-ventral extent, and reversed intestinal rotation (RIR) could affect the proper succeeding development of GI glands such as the pancreas (Pandya and Sutariya 2017). Additionally, RIR as reported in this case was associated with duodenal anomaly and absent of pancreas. These are one of the rarest forms of anomalies that have been reported. RIR associated with structural duodenal anomaly, absent pancreas, and lesser sac herniation implicitly denote to orchestrate whole gut development which is governed by genes products and molecular gradients. Another finding was unilateral cystic kidney. Multicystic kidney is the most common antenatally diagnosed cystic renal pathology. It refers to the presence of multiple renal cysts surrounded by dysplastic parenchymal tissue. This anomaly may be explained by an abnormal induction of metanephric blastoma by the migrating ureteric bud. It has been proposed that displaced metanephric blastoma interspersed with normal zones of nephrogenes generates the irregular parenchyma of the multicystic kidney. The subsequent cystic dilatation of dysplastic tissue compresses and permanently damages the normal renal tissue. GHARGE syndrome is a complex genetic syndrome which affects multiple system and organs. According to the Blake criteria (Blake et al. 2002), despite the sonography report indicating to CHRAGE syndrome, we could not find major criteria such as coloboma and choanal atresia. Also, due to some limitation, we could not find characteristic ear fine structures anomalies.