Extrahepatic portal vein obstruction (EHPVO) is a common cause of portal hypertensionin children yet very few studies are available. This study included 169 cases withEHPVO presenting to the Pediatric Hepatology Unit, Cairo University between 1996 and2010. The results of this descriptive study showed that the commonest presentingsymptom was hematemesis (58%). The diagnosis was made by ultrasound. Thecommonest possible risk factor was sepsis (6.5%). The most common clinical findingwas splenomegaly (83.4%). The liver functions were not affected. Upper gastrointestinalendoscopy was used for diagnosis and treatment of esophageal varices by injection orbanding. B blockers were used as the medical treatment to decrease portal pressure.