In seven children the clinical course of extrahepatic portal vein stenosis was studied angiographically. In all children with splenomegaly it was deemed necessary to perform an X-ray barium examination of the oesophagus. Transsplenic portal venography was performed when oesophageal varices were detected to set up the best scheme for emergency treatment. In our patients with hypersplenism there were no specific bone marrow alterations. In contrast to the literature most of our cases had fibrosis of the liver.