Spontaneous regression of hematologic malignancies is not uncommon and occurs in a wide variety of lymphomas and leukemias. In contrast, spontaneous remission of neurologic symptoms produced by these tumors is exceedingly rare. We report a patient with central nervous system acute lymphoblastic leukemia who experienced at least one spontaneous remission of papilledema and sixth nerve palsy. This represents, to our knowledge, the first case of spontaneous remission of neuro-ophthalmologic signs in a patient with acute leukemia. We conclude that meningeal leukemia may have a protracted course, and that spontaneous remission of neuro-ophthalmologic findings should not be so readily ascribed to a benign process in a patient with preexisting leukemia.