Anomalies of renal migration and fusion are rare. Ectopic kidneys that fail to ascend above the pelvic brim are commonly called pelvic kidneys. Bilateral cases have also been reported but are very rare. We present a case of 43 year old female who presented with intermittent dull aching pain in right lower abdomen, who had malrotated pelvic kidneys with vascular anomalies, associated with spina bifida. The majority of patients with ectopic kidneys are asymptomatic but those affected by obstruction, recurrent infection, or renal calculi, the anatomical position and associated anomalies should dictate the plan of management.