A 59-year-old Oriental male with chronic uveitis associated with vitiligo, poliosis, tinnitus and sunset glow fundus was diagnosed as having chronic Vogt-Koyanagi-Harada syndrome. He was treated successfully with steroids (systemic and sub-Tenon's) for two years, but then developed intractable vitritis and visual loss that worsened with the addition of azathioprine. A vitreous biopsy showed large B-cell lymphoma; a mass was also apparent in the subretinal space in the operated eye. Evaluation of the central nervous system was negative for lymphoma. Combined radiation treatment and chemotherapy resulted in dramatic resolution of the intraocular tumor. The possible role of immunosuppression in the development and progression of this tumor is discussed.