OEIS complex describes a combination of birth defects, including omphalocele, extrophy of the bladder, imperforate anus, and spinal defects. OEIS complex involves abnormalities of almost every body system and shows variability from case to case. The etiology of OEIS complex is thought to be multifactorial. The prenatal diagnosis of OEIS complex is difficult; however, the diagnosis of OEIS mainly relies on sonographic findings. The major sonographic findings include nonvisualization of the bladder, persistent cloaca, omphalocele, and myelomeningocele. Significantly increased maternal serum alphafetoprotein is also reported. Surgical intervention has improved the survival of patients born with OEIS complex; however, the success of these procedures cannot guarantee a good quality of life. The survivors may suffer with long- term psychosexual developmental difficulties.