Pilomatrixoma is a slowly growing benign tumor of the dermal hair cells. Metastatic disease is exceptionally rare. Pilomatrixoma can occur at any age, but most patients are older than 40 years at presentation. Approximately 60% of these lesions occur in the head and neck region. Their size is usually about 4 cm at the time of presentation. Surgical excision with adequate margins is still the preferred treatment. We report a case of an aggressive malignant metastatic pilomatrixoma in a 43-year-old woman who underwent multiple extensive local resections. However, she died within 4 months of presentation.