We report a case of malignant chondroid syringoma, a rare sweat-gland tumour with a propensity to occur on the extremities. These tumours must be distinguished histologically from their benign counterpart and from other malignancies with mixed epithelial and mesenchymal components. The malignant chondroid syringoma arises in the dermis or superficial subcutis and consists of eccrine or apocrine glandular epithelium, often showing mitotic activity and nuclear pleomorphism, within a benign-looking chondromyxoid stroma. It is locally infiltrative, with a tendency to local recurrence and metastasis. When the diagnosis is made histologically, clinical evidence of metastases should be sought. In the absence of metastatic disease local excision is curative.