Tracheo-oesophageal fistula, oesophageal atresia, and imperforate anus are known to be associated with renal anomalies - the VATER complex. The occurrence of ambiguous genitalia with this collection of anomalies has not previously been highlighted. We report two cases occurring 15 years apart; both had renal agenesis and neither was salvagealble from a renal or pulmonary viewpoint. Preoperative ultrasound examination of the renal tract should be performed in all babies born with the VATER complex and ambiguous genitalia; this will prevent unnecessary surgery.