Collecting duct carcinoma of the kidney is a rare variant of renal cell carcinoma. Hematuria is the most common presenting symptom. This tubulopapillary tumor arises from or differentiates forward to medullary collecting tubules. It is typically centered on the medulla of the kidney and extends into the cortex with a desmoplastic reaction. There are few reports documenting the clinical presentation and long-term prognosis. We describe a case of this uncommon tumor with clinical, histological and immunohistochemical features and review the literature.