Background: Vogt-Koyanagi-Harada disease (VKH), an inflammatory ocular disor der characterized by bilateral granulomatous panuveitis and a variety of extraoc ularmanifestations, has been reported to be associated with various immune disor ders but has not been linked to malignant lymphoma (ML). Case: We present here a case of VKH associated with a recurrence of ML. Observations: A 69-year-old m an who initially hadML presented with a history of sudden bilateral visual acuit y loss. Funduscopy showed papilloedema and serous retinal detachment in both eye s, and a diagnosis of VKH was reached soon thereafter. Chest X-ray and an abdom inal computed tomography scan indicated the metastatic focus of the ML. A recurr ence was suspected because the ML-associated soluble interleukin-2 receptor (s IL-2R) in the serum was highly elevated. Treatment successfully resolved both t he ML and the VKH. The inflammatory activities of VKH andML were found to correl ate with the serum levels of sIL-2R. Conclusions: This case suggests an associa tion between sIL-2R levels and disease activity in VKH andML, and provides addi tional evidence that VKH can be induced by immune disorders caused by high sIL- 2R levels in ML. Background: Vogt-Koyanagi-Harada disease (VKH), an inflammatory ocular disorbable by bilateral granulomatous panuveitis and a variety of extraoc ularmanifestations, has been reported to be associated with various immune disor ders but has not been linked to malignant lymphoma (ML Observations: A 69-year-old m an who initially hadML presented with a history of sudden or bilateral visual acuity loss. Funduscopy showed papilloedema and serous retinal detachment in both eye s, and a diagnosis of VKH was reached soon thereafter. Chest X-ray and an abdom in computed tomography scan indicated the metastatic focus of the ML. A recurr ence was missed because of ML-associated soluble interleukin-2 receptor ( Treatment of successfully resolved both ML and the VKH. The inflammatory activities of VKH and ML were found to correl ate with the serum levels of sIL-2R. Co nclusions: This case suggests an association between sIL-2R levels and disease activity in VKH and ML, and provides addi tional evidence that VKH can be induced by immune disorders caused by high sIL-2R levels in ML.