We report a case of syndromic gingival fibromatosis with notable ocular lesions, bilateral congenital cataracts, esotropia, and high myopia of a 21-year-old male patient from China. The patient was diagnosed with gingival fibromatosis based on his massive gingival overgrowth and histological findings that were consistent with gingival fibromatosis through a gingival biopsy. Lens opacity features were presented and phacoemulsificaion with intraocular lens(IOL) implantation was performed to manage the cataracts in both eyes. Transmission electronic microscopy was used to investigate the ultrastructure of the removed lens tissue. We also review the literature on gingival fibromatosis and briefly summarize the ocular manifestations of this rare disease.