A pilomatricoma is a rare, benign skin tumour originating from the hair follicle cells. It accounts for 0.15 of skin tumours is. It usually involves the head and neck region. The definitive diagnosis is based on histological examination. The recommended treatment is surgical removal of the lesion. Herein, we report a 40-year-old male case of pilomatricoma localized in the arm. We present our case due to its rare occurrence in the arm.