Pupose of Review: Pseudotumor cerebri syndrome (PTCS) may affect both children and adults, however the risk factors and clinical presentation vary greatly between these populations. This review aims to highlight the entity of PTCS in children and the unique considerations in this population; review the epidemiology and demographics; discuss the clinical presentation, revised diagnostic criteria, and approach to evaluation; review management strategies; and discuss the prognosis and long-term outcomes in children with PTCS. Recent Findings: Clinical presentation can be variable in children and may be less obvious than in their adult counterparts. Papilledema can also be challenging to diagnose in this population. The upper limits for opening pressure on lumbar puncture differ in children, with a cutoff of 25 cm H20 (or 28 cm H2O in a sedated or obese child). Summary: Morbidity related to visual loss, pain, and reduced quality of life lends urgency towards accurately identifying, evaluating, and managing children with PTCS. There are no randomised controlled studies to allow for evidence-based recommendations for the management of PTCS in children. Further studies are needed to clarify and consolidate management approaches in this population.