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Pilomatrix carcinoma was described in 1880 by Malherbe, is a rare and malignant adnexal tumor. It is a dermo-hypodermic tumor, with a low metastatic potential but a high risk of recurrence after excision. The average age of occurrence is 46.3 years. We report a case of a pilomatrix carcinoma of the head in a 15 years old patient. Pilomatrix carcinoma is a rare malignant tumor. It is usually reported in patients over 50 years. Nowadays, there is no standardized treatment while a high risk of recurrence after simple surgery was frequently observed. We report one case of a pilomatrix carcinoma in a 15 years old patient. In spite of a surgery with wide margins, she presented a local recurrence on the head and a lung metastasis. She had undergone a second surgery with additional radiotherapy and chemotherapy. She is now in remission.
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