Crossed fused ectopia is a renal congenital anomaly. It is remarkable for its associated anomalies in urogenital and other systems. Ectopic kidney occurs as a result of a halt in migration of kidneys to their normal locations during the embryonic period. Usually it is asymptomatic, but commonly the presenting feature is an abdominal lump. Proper knowledge of morphological variations of kidney and vessels supplying it are essential not only for anatomists but also for urologists. A sound knowledge of these variations assist in the diagnosis and management of renal anomalies. We present a case of unilateral ectopic kidney with polydactly managed in our centre.