Extrahepatic Portal Vein Obstruction is the most common cause of portal hypertension in children. However, it has a very low prevalence. Esophageal varices due to portal hypertension in children can lead to recurrent episodes of upper gastrointestinal bleeding, which can have a sinister outcome if timely diagnosis and treatment are not initiated.
A 7-year-old male child presents with recurrent episodes of upper gastrointestinal bleeding for 3 years. Clinical examination reveals pallor and splenomegaly. Laboratory investigations revealed signs of hypersplenism with anemia, leucopenia and thrombocytopenia, and Doppler ultrasonography and CT abdomen and pelvis revealed splenic vein thrombosis with splenomegaly and cavernous transformation of the portal vein. The patient was managed operatively with splenectomy with splenorenal shunting and devascularization of esophagogastric varices.
Extrahepatic Portal Vein obstruction is the most common cause of noncirrhotic portal hypertension in children. Its occurrence in the pediatric population is very rare. Portal hypertension can lead to variceal bleeding and splenomegaly, which can have a significant impact on a child’s long-term health. Because of its insidious nature, a meticulous workup is required for its diagnosis, and treatment in the pediatric population is difficult, and appropriate guidelines for its management specifically targeting the pediatric population are lacking.
Extrahepatic Portal Vein obstruction is rare in children with a difficult diagnosis and management. Despite these hindrances, timely intervention can lift a significant burden of its detrimental outcome off the young children and drastically uplift the quality of life of these patients.

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