A sixteen-year-old white girl with peripheral neurofibromatosis (NF1), who had been treated for a glioma of the optic nerves and chiasma developed a choroidal mass in her only functional eye. After a transchoroïdal biopsy, the pathologic examination disclosed a choroïdal melanoma of epithelioid cell type, using morphological criteria as well as an immunohistochemical study. After treatment with a ruthenium plaque, the tumor completely regressed over 4 months. According to the data of the literature, neurofibromatosis seems to predispose to the development of uveal melanomas.