Background: Melanocytomas of the optic disk are heavily pigmented beni
gn tumors that are most often discovered during routine examination, a
s they are classically asymptomatic. Although a slight impairment of v
isual acuity attributable to the tumor may occur in large tumors, acut
e profound visual loss is extremely unusual. Methods: A case involving
a 24-year-old woman with melanocytoma of the optic nervehead in the r
ight eye, who experienced acute severe visual loss, an afferent pupill
ary defect, and optic nervehead edema, is discussed. Laboratory tests
and serology for Toxoplasmosis, Syphilis, Borrelia, and Leptospira wer
e performed. Results: The severe loss of vision, optic disc swelling,
exudative detachment of the peripapillary retina, macular star, and sp
ontaneous resolution in an otherwise healthy patient were all consiste
nt with Leber's stellate idiopathic neuroretinitis. Results of serolog
ic testing were not contributory. Conclusion: This is the first report
of Leber's neuroretinitis associated with melanocytoma of the optic n
ervehead, and should be included in the differential diagnosis of neur
oretinitis. The possible mechanisms responsible for this association a
re discussed.