A 21-year-old Japanese woman was referred to our hospital because of severe anemia and thrombocytopenia. Bone marrow aspiration showed a hypercellular marrow with 91.5% promyelocytes. Cytochemical study and surface marker a diagnosis of acute promyelocytic leukemia. Because leukocyte count elevated, she was treated with all-trans retinoic acid (ATRA) after conventional chemotherapy. After 11 days of ATRA therapy, the patient started to develop severe headache, nausea and diplopia. Ophthalmologic examination revealed bilateral papilledema. Computed tomography and magnetic resonance imaging of the head showed no intracranial lesion. ATRA was discontinued because it was suspected to cause intracranial hypertension. Her symptoms were relieved and patilledema improved gradually. ATRA is safe and well-tolerated, if the retinoic acid syndrome can be prevented or managed. As the tolerable dose of ATRA in adults is higher than that in children, the side effects tend to occur in children. In Japan, only two childhood cases of intracranial hypertension during ATRA therapy have been reported. We must remember the possibility of intracranial hypertension during ATRA therapy, even in adults.

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