Congenital omphalocele with ectopic liver and hepatic cyst and unilateral kidney: A rare combination

Omphalocele is a rare congenital abdominal wall defect in which internal organs protrude through an abdominal wall defect covered by the peritoneum. There is paucity of literature on rare co-occurrence of ectopic liver in omphalocele content with congenital hepatic cysts. Here, we aim to report a case of a 33-week-old fetus with ambiguous genitalia and omphalocele. While performing an autopsy, we discovered that omphalocele contained an ectopic liver. In addition, we identified two hepatic cysts in the main liver and evidence of cholestasis on microscopy. Furthermore, a single kidney was identified in the sac. The condition was so clinically significant that it causes retardation of fetal growth and perinatal death of the fetus.