Chronic myeloid leukemia (CML) is a rare disease in children and adolescents and clinical presentations are variable . Symptoms are often attributable to anemia and splenomegaly. We present a case of secondary pseudotumor cerebri syndrome as the presenting feature in a pediatric patient with CML and a review of previous cases. Through these cases, we discuss this rare but important manifestation of chronic myeloid leukemia in pediatric CML, its cause and management. We conclude that pseudotumor cerebri is an important differential diagnosis in these patients as it may result in permanent visual loss if not addressed appropriately.