Purpose: To present a case of a young adult with bilateral chronic posterior uveitis resulting from Vogt-Koyanagi-Harada (VKH) disease treated with mycophenolate mofetil who subsequently developed vitreoretinal lymphoma in the right eye. Methods: A case and its findings were analyzed. Results: A 34-year-old Hispanic woman presented with an 8-year history of chronic recurrent posterior uveitis in both eyes secondary to VKH disease. A vitreous infiltrate that was treatment resistant occurred in the right eye. Subsequently, a diagnostic vitrectomy was performed and it was determined that the patient had developed unilateral vitreoretinal lymphoma. Conclusions: Masquerade syndromes should remain in the differential in patients with well-established uveitis who are on immunomodulatory treatment. A diagnostic vitrectomy should be considered when the treatment response is uncharacteristic. Furthermore, guidelines are lacking for central nervous system prophylaxis for isolated unilateral ocular intraocular lymphoma in young adults.