Renal and urinary tract anomalies have been reported to occur with a number of chromosomal abberations and a variety of müllerian developmental anomalies. Historically, the XY female has been privileged to remain free from these associated urologic complications. Presented are two types of the XY female in whom a major renal anomaly was found. It is possible that a potentially more frequent association of developmental anomalies has been overlooked. The urinary systems of XY females, when identified, should be investigated.