Crossed renal ectopia without fusion is a rare congenital anomaly in which one kidney crosses the midline and is located on the opposite side of its normal ureteric insertion. This condition is often asymptomatic but can be associated with complications such as hydronephrosis and ureteropelvic junction (PUJ) obstruction.

We report the case of a 29-year-old male with a known history of congenital crossed left ectopic kidney, congenital portal vein malformation, and colopathy, who presented with a six-month history of left lower abdominal pain. Imaging revealed progressive hydronephrosis of the ectopic left kidney without evidence of an obstructing stone or lesion. A Mag-3 renogram demonstrated impaired drainage (T ½ 33 minutes) and reduced split renal function (38% ectopic kidney). The etiology of the patient’s pain was unclear due to concurrent gastrointestinal abnormalities. A multidisciplinary team approach was undertaken, leading to a trial of ureteric stenting. Post-procedural improvement was mild, and the patient remains on an annual stent exchange plan.

Crossed renal ectopia is a rare anomaly, frequently associated with other congenital conditions. The management of symptomatic cases requires a multidisciplinary approach, particularly in patients with complex comorbidities. This case highlights the challenges in determining symptom etiology in patients with multiple congenital anomalies and underscores the need for individualized, patient-centered management strategies.

This case emphasizes the importance of multidisciplinary decision-making in managing complex congenital anomalies, particularly when differentiating between urological and gastrointestinal sources of abdominal pain. Further studies are needed to establish optimal management strategies for symptomatic crossed renal ectopia.