Collecting duct carcinomas are seen extremely rare. In this report, a 9 years old girl, who admitted to our clinic with hematuria and left flank pain for three months, is presented. Abdominal ultrasonography (USG), computed tomography (CT) and magnetic resonance (MRI) revealed a lobulated, hypoechoic, solid mass lesion The mass was 3x2.5 cm in diameters and located at the left renal pelvis level. Left transperitoneal radical nephrectomy was performed. The histopathological diagnosis was collecting duct carcinoma (Bellini) of papillary tubular type. Immunohistochemically, neoplastic cells had positive immunoreactivity to cytokeratine-7 (CK-7), HMWCK and CEA. Reevaluation of the patient, other organs involvement and local recurrence was not detected. Collecting duct carcinomas (CDC) are seen extremely rare and have poor prognosis and their diagnosis are difficult.