Myeloproliferative disorder, liver cirrhosis with portal hypertension, deficiency of natural anticoagulant proteins, gene mutation and hepatocellular carcinoma are the most frequent causes of portal vein thrombosis (PVT). Higher accuracy of the diagnostic methods is the reason why today the cause of PVT can be found more frequently. With imaging methods, PVT with or without cavernous transformation can be diagnosed. Fresh thrombus can be undetected in sonography due to the low echogenity but can be recognized in color Doppler sonography, especially with contrast-enhancing agent. Contrast-enhanced 3D MR angiography allows a comparable accuracy in the detection of PVT as digital subtraction angiography. Therapeutical options of PVT consist of mechanical recanalization of the portal vein, local fibrinolysis with or without placement of transjugular intrahepatic portosystemic stent shunt (TIPS), combination of mechanical recanalization and local fibrinolysis, systemic thrombolytic therapy, anticoagulation alone and surgical thrombectomy. Once PVT is found in sonography, Doppler sonography may be performed in order to distinguish benign from malignant thrombus. If further information is needed, MR angiography or contrast enhanced CT is the next step. If these tests are unsatisfactory, digital subtraction angiography should be performed. Until the early nineties, shunt surgery was recommended in patients with PVT who bled despite endoscopic treatment. Today, in symptomatic noncavernomatous PVT, recanalization with local methods is recommended. Additional implantation of TIPS should be performed when the patient is cirrhotic. In recent PVT in non-cirrhotic patients anticoagulation alone is recommended. It is expected that in old PVT anticoagulation can prevent further extension of the thrombus.