The experience with the management of pseudotumor cerebri at the Miami Children's Hospital in the past decade is presented. Twenty-two patients were identified; nineteen of which are included due to appropriate follow-up and data collection. No gender predominance was found and most cases were idiopathic without recurrences. Permanent visual loss did occur in some children in spite of surgical intervention with lumbar peritoneal shunt and optic nerve sheath fenestration. Pseudotumor cerebri in childhood does not present in the same fashion as in its counterpart adult and close follow-up is required to diagnose appropriately and intervene to prevent visual loss, which fortunately remains a rare occurrence.