Pilomatrixoma (calcifying epithelioma of Malherbe) is a rare, benign skin tumor derived from the outer sheath cells of the hair follicle. Pilomatrixoma has been reported not only as a benign lesion, but also as a highly malignant tumor. Basaloid (shadow) cells with or with no focal calcifications in shadow cell areas are typical. They occur predominantly on the face or neck, but may also arise on the extremities. We aim to report a case who presented with pilonidal disease located on the sacrococcygeal area. This is a very rare location for such a benign tumor. The ehoicey treetmont was surgical excision.