A 34 year-old man, with a two centimeter diameter quickly growing nodule on the auricular pavillion is presented. After mass excision the histologic diagnosis of pilomatrixoma was made. Pilomatrixoma is a rare benign tumor arising on the hair matrix. The differential diagnosis include pilomatrix carcinoma, a locally and recurrent aggressive tumor capable to make pulmonary metastasis. A review of the literature is included, with emphasis on the clinical and histopathological aspects and the therapeutic attitude.