Children with chronic liver disease or portal vein thrombosis frequently develop portal hypertension. The main complication of the portal hypertension are the development of esophageal varices who are at risk of variceal bleeding, which is associated with significant morbidity and mortality. Guidelines for adults with portal hypertension recommend performing esophagogastroduodenoscopy to identify those with varices that may benefit from prophylactic therapy, however there are no consensus in the pediatric age. This revision includes the incidence, diagnostic and the evidence for primary prophylactic treatment of the esophageal varices in children.