Ureterocele and ectopic ureter are the two main anomalies associated with complete renal duplication, but they also occur in a single system. At present, antenatal ultrasound detects both conditions in the majority of cases if associated with obstruction, and diagnosis is confirmed after birth by further examination. Later in life, these anomalies are revealed by clinical symptoms: urinary tract infections, pain, calculus formation, disturbances of micturition, and urinary incontinence. There is a wide variation of symptoms in patients with ureterocele (from the asymptomatic patient to urosepsis, urinary retention, and upper tract dilatation after birth). Diagnostic tools include ultrasound, voiding cystourethrography, magnetic resonance and radionuclide imaging, and cystoscopy. Management of both conditions comprises a conservative approach, endoscopic decompression, and surgical reconstruction. Choice of treatment is influenced by clinical symptoms, patient age, function of upper pole, presence of reflux or obstruction, and location of uterecocel/ureteral orifice.