A 56-year-old man complaining of blurred vision was referred to the ophthalmologist. He was initially diagnosed with uveitis and showed no response to steroid treatment. Diagnostic vitrectomy and skin lesion biopsy performed later confirmed the diagnosis of extranodal NK/T cell lymphoma (ENKTL), nasal type. Despite sufficient systemic chemotherapy, the patient developed central nervous system (CNS) relapse 3 months after achieving systemic remission. The diagnosis and treatment of this patient required multidisciplinary teamwork between ophthalmologists, pathologists, and hematologists. This report focused on the diagnostic planning and optimal treatment strategy for this patient.