We report a case of concomitant malignant fibrous histiocytoma (MFH) and tophaceous deposit at the second metacarpophalangeal joint in a 76-year-old man. The patient underwent surgical treatment, local radiotherapy and adjuvant chemotherapy and was disease free at the time of his last examination. We use this case to highlight the features of this clinical entity, which has never previously been described in the surgical literature.