Clinical and histopathologic findings in a case of localized congenital fibromatosis of the lower eyelid are presented. The histological and electron-microscopic features point to the hamartomatous origin of this tumor with partial differentiation of its cells towards myofibroblasts. In spite of the diffusely infiltrating growth and the tendency for local recurrences, metastases have never been observed. Therefore, local excision is the appropriate therapy.