BACKGROUND: Renal cell carcinoma nitric oxide synthase (NOS) rarely shows a rhabdoid morphology, and even more uncommonly in the chromophobe subtype. A case of chromophobe renal cell carcinoma (ChRCC) with a partial hemorrhagic pseudocystic appearance and a prominent rhabdoid component is reported. CASE: A 53-year-old man presented with microhematuria, and subsequently a 9-cm renal mass was discovered. Grossly, it was a largely hemorrhagic pseudocystic tumor with peripheral white solid masses corresponding to a ChRCC (NOS and eosinophilic subtype), dedifferentiated to a focal sarcomatoid and a prominent rhabdoid morphology, dyscohesive, sometimes in a myxoid stroma and in an inflammatory background, also present in the large hemorrhagic, necrotic, pseudocystic central area. Clinicopathology and immunohistochemical studies are described. CONCLUSION: There is no loss of SMARCB1 integrase interactor 1 expression in the rhabdoid component. Rhabdoid morphology is a dedifferentiated phenotype in different renal neoplasms, related to high grade, high stage, and poor outcome, like the present case, which highlights the importance of adequate sampling. © Science Printers and Publishers, Inc.