Benign fibrous histiocytoma (FH, dermatofibroma) is a common skin lesion but its metastasizing variant is extremely rare and only a few cases have been reported to date. The usual sites of metastases include locoregional lymph nodes and lung. In the majority of cases, the clinical course is indolent. At present, there are no reliable clinical or histological features of the primary tumour that could predict the risk of locoregional or distant metastases. Authors describe a case of metastasizing FH and briefly review available data.