We report a case of a young adult with an enormous mass lesion arising from the left kidney. His performance status was good despite severe constitutional symptoms. Radical nephrectomy was performed and histopathologi-cally it turned out to be an leiomyosarcoma. He made a good recovery. Surgical extirpation should be carried out to palliate the symptoms specially in young patients even though the prognosis is poor provided the patient has good performance status