A 12-year-old Caucasian boy developed leukemic hyphema with iris infiltration as the only relapse site during the third complete remission of his acute lymphoblastic leukemia. With high-dose methotrexate, high-dose cytosine-arabinoside plus teniposide, and a 5-week course of vincristine, prednisolone, and L-asparaginase, a complete remission could be achieved. Maintenance treatment was reinstituted for 1 year. However, after stopping the treatment, the iris infiltrate reappeared, and this time the eye was irradiated after chemotherapeutic reinduction. Seven months later, the boy remains in complete remission. The pathogenesis of leukemic iris infiltration is discussed briefly.