Inflammatory bowel diseases are associated with an increased risk of vascular complications. The most important are arterialand venous thromboembolisms, which are considered as specific extraintestinal manifestations of inflammatory bowel diseases.Among venous thromboembolism events, portal vein thrombosis has been described in inflammatory bowel diseases. We reportthree cases of portal vein thrombosis occurring in patients with active inflammatory bowel disease. In two of them, hepatic abscesswas present. Furthermore, we performed a systematic review based on the clinical literature published on this topic.