Portal vein thrombosis (PVT) is a common cause of portal hypertension in children. Predisposing conditions for PVT are obscure in more than half of the cases. Variceal bleeding and splenomegaly are the most frequent initial manifestations. Radiologic imaging studies are the mainstay for diagnosis. Treatment includes pharmacologic, endoscopic, and surgical modalities. β-Adrenergic blockers are not routinely used in children because of unproven efficacy and significant adverse effects. Endoscopic methods, such as sclerotherapy and endoscopic variceal ligation (EVL), are highly effective in the treatment of acute variceal bleeding and eradication of varices. EVL is the treatment of choice because of minimal complications and the need for few endoscopic sessions. EVL facilitates portal decompression either by the formation of collateral vessels or by surgical portosystemic shunting, when vessels grow to the proper diameter for anastomosis. Surgical portosystemic shunts are reserved for refractory cases because of significant complications and technical difficulties. Transjugular portosystemic shunts have an emerging role in the management of portal hypertension caused by PVT. PVT may occur in the posttransplant setting, but optimal management is not defined yet.