Congenital anomalies of the kidney and urinary tract (CAKUT) is an uncommon finding with challenging diagnosis establishment and potentially devastating long-term renal outcome. We describe a case of unilateral duplex collecting system with upper pole vesicoureteral reflux and lower pole ureterocele. This 4-year-old female with prior antenatal assessment of kidney abnormality presented with recurrent urinary tract infection. A multidisciplinary approach was employed to encourage comprehensive diagnostic workup and management strategy. The complexity of such a rare case warrants higher index of suspicion and coordinated collaboration of a multispecialistic team.