Renal cell carcinoma comprises over 90% of renal cancers, thus, it is the most common form of renal neoplasia. This carcinoma can often present itself in a variable fashion, ranging from incidentalomas to metastatic diseases. Furthermore, the most common metastasis associated with this type of carcinoma occurs in the lungs, bones or liver. We aim to report a case of renal cell carcinoma which presented together with a fibromixoid sarcoma.A 50 year-old woman presented with hematuria, weight loss, asthenia and right lumbar pain that started 7 months prior to the consult. CT scan was performed and revealed a solid injury of 10 cm in the right kidney together with a mass in the left flank. Total right nephrectomy was promptly performed and the patient was submitted 3 months later to a tumoral resection of the abdominal wall. Histopathological findings revealed a primary renal cell carcinoma and the second, metastatic tumor was shown to be a fibromixoid sarcoma. The patient was not submitted to chemotherapy and is currently under follow-up with the surgery and oncology staffs, without showing any symptoms.Renal cell carcinoma usually presents itself together with secondary tumors on the lungs and bones. The association of this type of carcinoma with a fibromixoid sarcoma of the abdominal wall is rare and poorly reported in the literature.This case reports shows a successful treatment regarding this rare association, which can help other physicians to re-evaluate their medical conduct.