Bilateral uveitis, poliosis, hypomelanosis, and alopecia (Vogt-Koyanagi-Harada syndrome) developed spontaneously in a 57-year-old woman following operation for metastatic malignant melanoma. She has been disease-free for 8 1/2 years in spite of a highly unfavourable prognosis. Within the framework of current concepts of cell-mediated and humoral immunity in patients with malignant melanoma, activity of the host's immune system is postulated for the destruction of normal melanocytes in the skin, hair follicle, and uveal tract, as well as for the favorable outcome.