A case is presented wherein a set of black female pygopagus siamese twins were successfully separated by our department in their 45th day of life. Both twins had multiple malformations: cardiopulmonary, vertebral, anorectal, genitourinary, etc. The bilateral renal absence in one of them, originated her death on the 7th postoperative day. Only nine other cases have been referred successful during the last 35 years, in a bibliographic review conducted. The associated malformations and surgical skills for treatment and separation of them, are studied: previous colostomy and effective separation at life ages, comprised between one and thirteen months.