Objective To summarize the experience in diagnosis and treatment of portal cavernoma in children and inquire its prognosis of different therapies.Methods Analysing the diagnosis and treatment effect on 9 cases with portal caver cavernoma aged 1～11, 7 cases were followed up. Results The first noticed symptoms showed upper digestive tract hemorrahge and splenomegaly. The postive rate of diagnosis of portal cavernoma by ultrasound was 88.9% and false postive rate was 2.3%. The patients under the operation of portacaval shunt and splenectomy had ≤1 episode of upper digestive tract hemorrage per year, which had better outcome than those under only splenectomy or portacaval devescularization and splenectomy. Conclusions The possibility of portal cavernoma should be considered in children with portal hypertension.Ultrasound can be used as an ordinary method in the diagnosis of the cases. Portacaval shunt and splenectomy is the first choice management in the patients when condition is permited.