Pilomatrixoma, also known as calcified epithelioma of Malherbe, is the most common tumor of the hair follicle. It is a benign tumor in a young adult. Pilomatrixoma is most commonly located in the head and neck. It is usually misdiagnosed and confused with other skin lesions. The authors report a rare localization of this tumor in the left hand in a 40-year-old patient treated surgically without recurrence after four years of follow-up.