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