Chromophobe renal cell carcinoma with liposarcomatous dedifferentiation is a very extremely rare tumor and only few cases have been reported in the literature. Here we report a case of a 37-year-old woman who presented with a pain and a palpable mass in the right flank. The abdominal computed tomography (CT) scan found a renal tumor and the patient underwent a right radical nephrectomy with adrenal gland resection. After the histological examination of the specimen completed by immunohistochemical and molecular study, a diagnosis of chromophobe renal cell carcinoma with liposarcomatous dedifferentiation was made. The patient received adjuvant chemotherapy. Afterwards, she developed bone metastasis and died 13 months after the surgery. Chromophobe renal cell carcinoma with liposarcomatous dedifferentiation is a rare tumor associated with a poor prognosis and a metastatic potential.