This is a case of a 38yr old male who presented with a history of a slowly growing scalp lesion initially noticed about 10 years ago. The mass was painless, and the patient has no constitutional symptoms. There was no preceding history of trauma. Plain radiograph of the skull showed a lobulated mass of soft tissue density in the left parietal scalp region close to the vertex. The underlying skull vault was intact. Cranial computed tomographic (CT) scan revealed a well-defined solid, lobulated mixed density mass within the skin and subcutaneous tissue in the left parietal region close to the vertex with multiple coarse calcifications within it; the mass showed heterogeneous contrast enhancement. No intracranial extension of the lesion was seen and there was no evidence of vault destruction. Histology following incisional biopsy revealed a rare proliferative trichilemmal tumor.