Proliferating pilomatricoma is a distinctive rare variant of pilomatricoma first described in 1997.Very few cases of proliferating pilomatricoma have been reported in the literature until date. This report is a case of 65 years old male presenting with hard nodular lump on the right side of neck slowly increasing in size for the last 8 months. Wide excision of the lump was performed with clear surgical margins. Histopathology revealed a tumor mass composed of lobular proliferation of basaloid cells in association with adjacent focal areas containing cornified material with shadow cells. Variable nuclear atypia and scattered mitotic activity was noted in basaloid cells, so a final diagnosis of proliferating pilomatricoma was rendered. This report herein reviews histopathological features of this rare entity.