Congenital portosystemic venous shunt is extremely rare and should be treated. Advances in treatment techniques allow for patients to be treated safely. We present a 9-year-old boy with a large congenital portosystemic venous shunt. The shunt was occluded interventionally with the Amplatzer vascular plug II. Our case was unique with its clinical manifestation, the use of a 22-mm Amplatzer vascular plug II, and the presence of the patient’s 1-year follow-up.
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