A case of complete duplication of the left femur is presented. This anomaly was associated with ipsilateral tibial hemimelia, lumbar myelomeningocele, extrophy of the cloaca, ambiguous genitalia, imperforate anus, and omphalocele. The constellation of anomalies implies an insult at about the fourth week of gestation. Maternal ingestion of hydroxyzine was reported in this case. Management of the limb anomaly was by resection of the duplicate femur and malformed leg. Preoperative arteriography was found helpful.