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An unusual case of malignant cylindroma of the scalp arising in a 79-year-old white female with multiple cylindromatosis is presented. The tumor apparently arose from a cylindroma and had features of spiradenoma. Multiple cylindromatosis is an uncommon hereditary autosomal dominant disease, which is characterized by multiple skin adnexal tumors like cylindromas and trichoepitheliomas and occasional spiradenomas. Cylindroma is an uncommon benign tumor, which originates from skin appendages and is most commonly found on the scalp and face with a strong predilection for middle-aged and elderly females. Although cylindromas are usually benign neoplasms, carcinoma arising in such neoplasms is rare with only sporadic reports in literature. Her family history was negative for a similar disease. The patient's main concern was painful lesions over her right ear that interfered with wearing of her glasses. The clinical, histological immunohistochemical features, and treatment are presented along with a review of the literature.

Malignant Cylindroma of the Scalp Arising in a Setting of Multiple Cylindromatosis: A Case Report