Pilomatrix carcinoma is the rare malignant counterpart of pilomatrixoma, a skin adnexal tumour arising from hair matrix cells that arises either as malignant transformation of pilomatrixioma or as de novo. Pilomatrixoma was first thought of having a sebaceous gland origin but was later found to originate from hair matrix cells. They are slow growing tumours, found in the lower dermis and subcutaneous fat and are predominantly found in the neck and the scalp region. They are rare tumours with 90 cases reported so far.