The partial or complete obstruction of portal vein and or one of its branches is the most frequent cause of portal hypertension in children. A patient with extrahepatic portal hypertension and progressive course is presented, with great development of esophagogastric varices and multiple episodes of upper gastrointestinal hemorrhage. At the age of 9 years he was submitted to an azygos-portal disconnection by abdominal route, with manual transection of the esophagus, covered and protected by a gastric fundoplication. Postoperative endoscopic control demonstrated significant reduction in the size of esophageal varices and disappearance of gastric varices. Eleven months after surgery the patient was asymptomatic and a new endoscopic control showed no recurrence of varices.