Retrocaval ureter is a rare congenital vascular anomaly with an incidence of 0.13%, leading to the passage of the right ureter behind the inferior vena cava and then turning around it to attain its lateral position. The condition is usually associated with obstruction in the right kidney and proximal ureter leading to symptoms like dull aching pain in the flanks, recurrent episodes of urinary tract infections, and recurrent stone formation. The patient presented with recurrent episodes of burning micturition and pain in the right flank for the past 6 months. A contrast-enhanced computed tomography kidney–ureter–bladder was done to diagnose the condition. The patient was managed by open pelviureteric anastomosis lateral to the inferior vena cava, thus eliminating the obstruction on the ureter. The patient has had an uneventful postoperative follow-up. Retrocaval ureter is a rare condition and should be clinically suspected in cases of hydronephrosis where other causes have been ruled out. Different approaches can be used to correct the anomaly. In this case report, an open transperitoneal intraabdominal approach has been used.