Epigastric heteropagus associated with an omphalocele and double outlet right ventricle

•We diagnosed epigastric heteropagus prenatally by ultrasonography and MRI.•Preoperative CT angiography was valuable for surgical separation.•Common associated anormalies are omphalocele and cardiac malformations.•Prognosis and treatment largely depend on the cardiovascular system.

Incomplete or asymmetrical conjoined twins are extremely rare congenital anomalies. We report a case of epigastric heteropagus associated with an omphalocele and double outlet right ventricle. The cystic legion of the epigastrium was detected in our patient by an ultrasound scan at 28 weeks' gestation. He was born at 37 weeks' gestation by scheduled caesarean section. A parasite with an incomplete head and lower limb was attached to the epigastrium of the autosite. Surgical separation of the parasite and silo placement for an omphalocele was successfully performed on the 4th day of life. He underwent secondary surgical closure of the omphalocele on the 10th day. For treatment of the cardiac anomaly, he underwent an operation of Blalock–Taussig shunt because of pulmonary artery stenosis at the age of 3 months and correction of double outlet right ventricle at the age of 10 months. At the 20-month follow-up, he was alive and showed a normal growth pattern.