A ~corkscrew deformity~ of the proximal ureter is a rare congenital anomaly that typically lacks any postnatal clinical significance. The rarity of this entity, however, has not allowed the clarification of its natural history and the ideal approach to its management. We herein present a case of a 27-year-old female patient who presented with right flank pain and significant hydronephrosis. On retrograde ureterography, a typical spiral configuration of the proximal ureter was noted. The patient underwent successful reconstruction by laparoscopic transperitoneal dismembered pyeloplasty. We report the first use of laparoscopic reconstruction for the management of ~corkscrew deformity~ of the proximal ureter and we focus on the imaging findings, technical details, advantages and limitations of this technique.