Pseudotumor cerebri (PTC), also known as idiopathic or benign intracranial hypertension, is characterized by elevated intracranial pressure without any evidence of organic central nervous system disorders. PTC is a rare but well-known adverse event associated with all-trans retinoic acid (ATRA) treatment. Persistent blindness in about 10% of affected patients is caused by PTC; therefore, prompt and accurate diagnosis is required. Recently, magnetic resonance imaging (MRI) findings, including empty sella, flattening of the posterior aspect of the globe, and distention of the perioptic subarachnoid space, have been found to be of high diagnostic value in PTC, but there apparently have been no reports about MRI findings in ATRA-induced PTC. We report here an 11-year-old boy with acute promyelocytic leukemia who developed ATRA-induced PTC during leukemia treatment. MRI findings beneficial for the diagnosis of PTC were observed.