Chondroid syringoma is a rare, skin appendageal tumor. It is also known as mixed tumor of skin, as it histologically resembles mixed tumor of salivary gland (pleomorphic adenoma). It is most commonly a benign tumor, but a few malignant counterparts have been described in history. It usually presents as a solid, slow-growing, solitary, and painless nodule in the head and neck region. Malignant counterpart is rare and commonly affects trunk and extremities. Early diagnosis and surgery by wide local excision are the most reliable treatments to date. Recurrences are common and hence close follow-up is advised. In this study, we present a case of malignant chondroid syringoma of face with extensive extension into nose and paranasal sinuses, which was recurrent and managed by surgical excision and radiotherapy. To the best of our knowledge, this is the first reported case with extension and bone destruction into nose and sinuses.
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