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Acute portal vein thrombosis (PVT) is a relatively rare diagnosis with a nonspecific clinical presentation. Imaging plays an important role in establishing the diagnosis as well as the etiology and complications of acute PVT. Prompt diagnosis is essential to prevent catastrophic short-term complications including bowel infarction, sepsis, and possible death; missed diagnosis can also result in the long-term sequelae of portal hypertension. Differentiation of acute from chronic PVT is crucial as management strategies differ. Currently, guidelines for treating acute PVT recommend immediate initiation of systemic anticoagulation. Catheter-directed therapy may be used in combination with systemic anticoagulation in the setting of bowel ischemia or as an adjunct in patients with a contraindication to systemic anticoagulation. In this review article, we discuss the diagnosis and clinical features of acute PVT, focusing on current medical and endovascular management strategies including mechanical thrombectomy and fibrinolytic therapy.