A 21-year-old woman with neurofibromatosis type 1 (NF-1) had a unilate
ral congenital Horner's syndrome with resultant hypopigmentation of th
e affected iris. Lisch nodules, which are melanocytic hamartomas, were
similar in number, size, and pigmentation in both eyes. The present f
indings suggest that the formation of Lisch nodules is not influenced
by the presence or absence of sympathetic innervation of the iris.