Abstract We report a rare tumor called low-grade renal collecting duct carcinoma. Grossly, the tumor consisted of multiple cysts and solid white nodules, measuring 10 cm in diameter and occupying most of the renal parenchyma. Histologically, the tumor was characterized by well-differentiated tubules lined by eosinophilic cells without papillary projections, abundant predominantly extracellular mucin, minimal cellular atypia, no desmoplasia, and rare mitoses. This tumor occurs in collecting ducts and the tumor cells were positive for epithelial membrane antigen, high-molecular-weight keratin, CD15, and mitochondrial antibody and negative for CD10. Few cells stained weakly positive for ulex europaeus. Ultrastructural study showed a large number of mitochondria according to the eosinophilic cells seen in light microscopy.