This observational cohort study reports the short- and long-term clinical outcomes of 31 patients admitted for acute non-malignant, non-cirrhotic portal vein thrombosis (PVT) over a 10-year period. Patients had a mean age of 43 years at admission and a mean duration of follow-up of 84 months. All patients were initially treated with anticoagulants. Complete recanalization occurred within 30 days after admission in 18 patients (58%), partially in nine patients (29%), and failed in four patients (13%). During follow-up, 10 patients (32%) had at least one episode of gastrointestinal bleeding. The probability of remaining bleed-free was 0.93 at 24 months and 0.61 at 48 months. Fundal varices were not controlled by endoscopic sclerotherapy, so all four patients underwent portosystemic shunt construction. To date, there has been no mortality. In conclusion, using a combination of different treatment options reduces the risk of death and late complications in patients with non-malignant, non-cirrhotic PVT.