Pseudotumor cerebri or idiopathic intracranial hypertension is a neurological syndrome characterized by signs and symptoms of intracranial hypertension without clinical and radiological evidence of infective or space occupying lesions. Iatrogenic factors are frequent; in particular, cases of Pseudotumor cerebri associated with all-trans-retinoic acid treatment in acute promyelocytic leukemia (APL) have been frequently described in pediatric patients. We review the literature and give diagnostic and therapeutic guidelines.