Omphalocele, exstrophy of the bladder, imperforate anus and spinal defect (OEIS) complex is a rare congenital multisystemic malformation representing unique anomalies. It was first reported in 1978 through a series of cases with an abnormality of body wall development. We are reporting a case of an infant of 36 weeks gestation, with a family history of consanguinity and oral contraceptive pill intake that was discontinued when the mother was 1-month pregnant. The neonatal examination revealed findings that were consistent with OEIS complex along with the presence of genital anomalies. The infant required multi-staged surgical intervention. We conclude that this case report might illustrate some of the possible risk factors and variability of OEIS complex.