The authors report the autopsy case of a 73-year-old man with widespread, metastatic, malignant chondroid syringoma, with a long clinical history. Three years after resection of the primary tumor on the back, the tumor recurred at the same site, metastasized to the cervical lymph nodes 7 years later, and finally, after 13 years exhibited widespread metastases. Histologically, the tumor showed cords and nests of cuboidal or polygonal tumor cells with little cellular pleomorphism and few mitoses surrounded by a mucoid matrix. Histochemically, the matrix contained hyaluronic acid and sulfated acid mucopolysaccharides, such as chondroitin sulfate A and/or C. To the knowledge of the authors there have been nine reported cases of malignant chondroid syringoma in the English literature, four of which had distant metastasis. A review of the literature reveals that malignant chondroid syringoma is more common in women and occurs most often in trunk and extremities, which is in contrast to its benign counterpart, its histologic appearances vary greatly, and it may even have benign appearances.