Portal vein thrombosis is a rare condition seen in the general population, though is more prevalent in certain populations with thrombophilia as well as in patients with cirrhosis. Because morbidity and mortality can be significant with portal vein thrombosis, diagnosis and appropriate management are crucial. In noncirrhotic patients, untreated portal vein thrombosis can result in mesenteric ischemia and complications of portal hypertension. In cirrhosis, portal vein thrombosis is often incidentally found on routine screening imaging, and the treatment outside of transplant candidates is more controversial. In this review, we will outline risk factors for portal vein thrombosis, discuss indications for treatment, and evaluate both pharmacological and endovascular treatment options, highlighting unique clinical considerations dependent on the presence or absence of cirrhosis.