A 75-year-old man presented with a protuberant stable lesion of many years on his left second toe. Histology on an incisional biopsy showed myxoid pleomorphic fibroma. Over the subsequent 21 months, the lesion enlarged and became exophytic and irregular. Repeat biopsy showed features consistent with a grade III myxofibrosarcoma. Treatment involved amputation of his toe. Six months later he remains well, with no evidence of local recurrence or systemic spread.