ObjectiveTo discuss etiology, pathology, clinical manifestations, diagnosis, management and prognosis of cavernous degenerarion and hypertension of portal vein.MethodsThe clinical and image data of 7 children with cavernous degeneration and hypertension of portal vein were veviewed.ResultsHematemesis, melena and splenomegaly were the major presentations. The diagnosis was confirmed by color Doppler ultrasonography except 4 cases had angiography. All cases underwent splenectomy and transection of the gastric fundus and esophageal veins. Five of them had an additional portalcaval shunt.ConclusionCongenital factors may play some role in the development of cavernous degeneration and hypertension of portal vein in children. For the disase discussed this paper the image examination is the main diagnostic method, and the management is same with portal hyertension and the prognosis is closely related to the liver function.