Chondroid syringoma (CS) (mixed tumor of the skin) is a rare neoplasm of the sweat glands, which presents itself as a slow-growing, painless, nonulcerated, subcutaneous or intracutaneous mass often occurring in the head and neck region. The clinician may miss the diagnosis of this lesion due to its rarity. CS should be considered in the differential diagnosis of any subcutaneous nodules, especially in the head and neck region. The diagnosis of CS is mainly based on the histopathologic examination. This article presents a 35-year-old male with a mass on the upper lip that was histopathologically diagnosed as an apocrine variant of benign CS with squamous metaplasia after surgical excision. No sign of recurrence is evident till date during the follow-up. We report this case because of its rarity. A brief literature review and all the reported cases in the lip have been listed.

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