Malignant transformation of solitary enchondromas of the hand to secondary chondrosarcomas is extremely rare. We report a case of a recurrent chondromatous tumor of the hand that initially presented with the typical histology of a cellular enchondroma of the small tubular bones but with clinical and radiological signs of malignancy. After development of a single pulmonary metastasis of a chondromyxoid tumor a malignant transformation of the primary enchondroma of the hand must be assumed.