Portal hypertension should be considered in a patient with signs such as ascites, hypersplenism, or thrombocytopenia. Etiologies are broadly classified into cirrhotic and noncirrhotic etiologies. The noncirrhotic etiologies are classified into prehepatic, intrahepatic, and posthepatic causes. Treatment is determined by etiology: sinistral portal hypertension can be treated with splenectomy, portal venous thrombosis can be treated with anticoagulation, and parasitic infections can be treated with antibiotics. In select patients with hepatic outflow obstruction, mesenteric shunts can be created if there is no evidence of liver failure. Often, treatment is directed at symptom control, with management of gastrointestinal bleeding or ascites. Patients with progressive liver failure should be referred to liver transplantation unless there is a specific contraindication.