To present a case of leukemic infiltration of the optic nerve head as the initial manifestation of leukemic relapse.

A 65-year-old woman was diagnosed with acute myeloid leukemia. Complete remission was achieved after 4 complete courses of chemotherapy. She complained of a sudden decrease in visual acuity in her left eye. Fundus examination showed severe optic disc edema with peripapillary hemorrhage and serous retinal detachment. Visual acuity and fundus continued to aggravate and high-dose intravenous steroid therapy was instituted. Visual acuity and fundus deteriorated more after treatment. Brain magnetic resonance imaging and CSF study were normal but intrathecal chemotherapy and focal irradiation were performed on account of the suspected CNS involvement of leukemia. Morphologic improvement in the retinal structure was achieved, however, optic atrophy remained and her vision did not recover.

The present case shows the involvement of the optic nerve head as the initial isolated manifestation for the relapse in a patient with complete remission. CNS involvement is rare in acute myeloid leukemia and in particular, the optic nerve is rarely reported as the initial isolated presentation for the relapse. Moreover, the disease progression relatively aggravated after treatment. In the atypical aspects of leukemic relapse, the present case was noticeable.