A case of absences of external genitallia, urethra, rectum, anus, and defects of bladder, colon with omphalocele was presented. This absences of external genitallia, urethra, rectum, anus, and defects of bladder, colon with omphalocele is a rare congenital malformation of a serious nature. This malformation is due to environmental defects, chromosomal anomalies and inborn errors of metabolism. In this malformation the prognosis is extremely poor. A review of literature was made.