Ureterocele is a cystic dilatation of the intravesical ureter that is most commonly observed in females and children, and usually affects the upper moiety of a complete pyeloureteral duplication. According to their position, ureteroceles are divided into intravesical, when the ureterocele is completely contained inside the bladder, and extravesical when part of the cyst extends to the urethra or bladder neck. Most ureteroceles are diagnosed in utero or immediately after birth during an echographic screening of renal malformations. Severe, febrile urinary tract infection is the most common postnatal presentation of ureteroceles, but they may, rarely, prolapse and acutely obstruct the bladder outlet. Once an ureterocele is identified sonographically, a voiding cystourethrogram to detect vesicoureteral reflux (VUR) and a 99m-technetium dimercapto-succinic acid renal scan to evaluate the function of the different portions of the kidney are mandatory. VUR in the lower pole is observed in 50% of cases and in the contralateral kidney in 25%. Simple endoscopic puncture of the ureterocele has recently been advocated as an emergency therapy for infected or obstructing ureteroceles and as an elective therapy for intravesical ureteroceles. The rate of additional surgery after elective endoscopic puncture of an orthotopic ureterocele ranges from 7 to 23%. Treatment of ectopic ureteroceles is more challenging and both endoscopic puncture and upper pole partial nephrectomy frequently require additional surgery at the bladder level. The reoperation rate after endoscopic treatment varies from 48 to 100%. It is 15 to 20% after upper pole partial nephrectomy if VUR was absent before the operation, but is as high as 50-100% when VUR was present. Thus, endoscopic incision is appropriate as an emergency treatment or when dealing with a completely intravesical ureterocele. Upper pole partial nephrectomy is the elective treatment for an ectopic ureterocele without preoperative VUR. In an ectopic ureterocele with VUR, no matter which type of primary therapy has been chosen, a secondary procedure at the bladder level, involving ureterocele removal and reimplantation of the ureter(s), should be anticipated.