Myxofibrosarcoma, previously known as malignant fibrous histiocytoma, myxoid variant, is a rare tumor of mesenchymal origin, composed of spindle cells and myxoid stroma. It mainly affects elderly people, involving the lower extremities and frequently extending to the dermis and subcutaneous tissue. The tumor presents high rates of recurrence, and a deep biopsy is required to obtain the correct diagnosis. We report a case of high-grade mixofibrossarcoma characterized by a rapidly growing tumor and the presence of marked cellular pleomorphism and an abundant myxoid matrix.