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The aim of this study is to analyze the incidence, risk factors, management, and follow-up of patients with portal vein thrombosis (PVT) undergoing primary orthotopic liver transplantation (OLT). Four hundred fifteen OLTs were performed in 391 patients. In 62 patients, partial (group 1; n = 48) or complete (group 2; n = 14) PVT was found at the time of surgery. Portal flow was reestablished by venous thrombectomy. In this study, we compare 62 primary OLTs performed in patients with PVT at the time of OLT with a group of 329 primary OLTs performed in patients without PVT (group 3) and analyze the incidence of PVT, use of diagnostic methods, surgical management, and outcome. We found no significant differences among the 3 groups for length of surgery, cold and warm ischemic times, and postoperative stay in the intensive care unit. With the piggyback technique, groups 1 and 2 had greater blood losses and required more blood transfusions than group 3. The early reoperation rate was greater in group 2. The incidence of rethrombosis was 4.8% (group 1, 2%; group 2, 14.3%). Reexploration and thrombectomy (2 patients) and retransplantation (1 patient) had a 100% mortality rate. In particular, the mortality rate of patients with complete PVT with extension into the splanchnic veins is high (33%). Three-month and 4-year patient survival rates were statistically similar in the 3 groups. The presence of PVT at the time of OLT is not a contraindication for OLT. However, if PVT extends into the splanchnic veins, the outcome is guarded.