BackgroundBilateral single system ectopic ureter is a rare congenital anomaly, but bilateral ureteral ectopia with duplex collecting system with bladder hypoplasia is even rarer. Urinary incontinence in such cases is dealt with various reconstructive procedures.Case presentationA 20-year-old female presented with continuous urinary incontinence without normal voiding since birth. Imaging revealed bilateral duplex collecting system with ectopic ureter draining into the vestibule, and hypoplastic bladder. The patient was managed with Mainz-II pouch creation with serous lined extramural ureteral reimplantation technique with complete resolution of incontinence.ConclusionOur article highlights the challenges in surgical planning, treatment and the patient perspectives for this rare congenital malformation presenting with incontinence.