Congenital absence of the umbilical cord resulting from maldevelopment of embryonic body folding

We report a case of extreme maldevelopment of embryonic body folding that resulted in incomplete fusion of the amnion to the chorion and failure to form an umbilical cord. Massive intra-abdominal and intrathoracic herniation led to rapid neonatal death. The accurate ultrasonic characterization of the defect enabled prudent perinatal management.