We present the case of a young man with Klinefelter syndrome, who was admitted to our clinic with renal colic. Shortly after admittance, spontaneous decrease in pain has occurred. Ultrasound and intravenous contrast computed tomography were performed, which showed the evidence of urine extravasation at the level of left renal pelvis and a 4 mm stone in the lower third of the left ureter. The management with a double-J ureteric stent for three weeks was successful. Then, the stent was removed and computed tomography confirmed the absence of urine extravasation. We also analyze the literature related to this case and discuss the main mechanisms of collecting system rupture.