Cases of adult renal cell carcinoma with rhabdoid features have been reported in the literature, usually in association with conventional clear cell and papillary tumors. Till date, only one report described chromophobe renal cell carcinoma with rhabdoid differentiation. We report a case of a 47-year-old male patient that underwent a routine medical check-up. Computer tomography scan revealed a large heterogeneous mass in the left kidney, multiple nodular changes in the lungs and multiple enlarged lymph nodes. On microscopic examination of the left nephrectomy specimen, 65 % of the kidney tumor was comprised of rhabdoid foci and the rest of the tumor showed eosinophilic areas consistent with chromophobe renal cell carcinoma. Final diagnosis was chromophobe renal cell carcinoma with rhabdoid differentiation. Behavior of these tumors in adults is more aggressive, demands more aggressive treatment and has poor prognosis. Therefore, rhabdoid differentiation should be recognized in this type of carcinoma and mentioned in the pathohistology report.