Abstract: A case of onychomatricoma was reported and related literature was reviewed. A 54-year-old male presented with 10-year history of thickening and xanthonychia of nail plate on the right second fingernail. Histopathological examination showed the nail plate thickening, proximal matrix cells V-shaped proliferation, distal zone papillary projection along nail plate covered by eosinophilic keratogenous zone, exudation and crusting on part of tip of papilloma, spindle cells accumulated with scattered mast cells in upper dermis, in deep dermis fibres and fibrocytes proliferated that arranged parallel to the epidermis. Immunohistochemistry showed CD34 positive in spindle cells, S-100, F XIIIa, CD68, and CK7 negative. The diagnosis of onychomatricoma was made. The patient was treated with complete surgical excision of the tumor with no recurrence after 10 months follow-up.