Ocular complications of chronic active Epstein-Barr virus infection (CAEBV) have rarely been reported. We describe a 7-year-old girl with CAEBV with associated uveitis. The patient was first observed to have recurrent fever and hepatos-plenomegaly in August 1991. She presented with left facial nerve palsy in June 1993. Ocular examination showed right iridocyclitis. Both optic disks were swollen, and the retinal vessels were dilated. Antibody titers to EBV were markedly elevated. Treatment with topical steroids, systemic interleukin-2 and splenectomy dramatically relieved all her symptoms, including the ocular ones.