Portal hypertension (PH) is a common complication of chronic liver disease in children and represents a cause of morbidity and, rarely, mortality in this group of patients. Although often self-limiting, gastrointestinal bleeding in this setting is regarded as a frightening event by patients and carers, giving the impression of impending death. Therefore, it is important to raise the awareness on the natural history of PH in children, the utility of tools that help preventing and managing acute bleeding, and the signs predicting a poor outcome, thus indicating surgery. There is lack of data on the ability of endoscopy screening, endoscopic treatment of varices, and use of nonselective β-blockers to alter the outcome of PH in children; major efforts should be made to avoid such treatments empirically and promote multicenter trials instead. Nevertheless, such approach should be balanced against the need of offering the best care to children with PH. In this review, we discuss the advances made in the management of PH in children and compare it with the larger adult experience. A rational approach to acute gastrointestinal bleeding is proposed along with an algorithm suggesting a stepwise protocol to manage children with esophageal varices in the long-term, with some hints on possible future studies.