We endeavored to determine the frequency and clinical characteristics of portal hypertensive gastropathy (PHG) in children with cirrhotic and noncirrhotic portal hypertension. Medical records of all patients with portal hypertension undergoing upper GI endoscopy during a 7-year period were retrospectively reviewed. PHG was defined as absent, mild, or severe by previously established criteria. Twenty-two patients with portal hypertension (17 cirrhotic, 5 noncirrhotic) were identified. In the group of 17 cirrhotic patients, PHG was noted in four at initial endoscopy (three mild, one severe) and in seven (two mild, five severe) during follow-up. Severe PHG was seen in one patient with noncirrhotic portal hypertension at presentation; in two patients, mild PHG developed during follow-up. In the 14 patients in whom PHG was noted, gastric mucosal disease developed in nine in whom no sclerotherapy was performed and in five patients treated with sclerotherapy. The size of varices, history of variceal bleeding, and presence or absence of hypersplenism did not appear to be related to the development of PHG. None of seven patients with mild PHG and two of seven patients with severe PHG bled from their gastric mucosa. We conclude that PHG is commonly observed in children with cirrhotic and noncirrhotic portal hypertension. Mild PHG appears to have little clinical significance, while severe PHG may cause bleeding. PHG may develop with or without sclerotherapy.