Objective We sought to review the etiopathogenesis, diagnosis, and surgical options for 253 patients with portal vein thrombosis (PVT) undergoing orthotopic liver transplantation (OLT) to assess the the impact of PVT on outcomes. Methods We retrospectively analyzed the data from 2508 adult patients undergoing 2614 OLTs in our center from September 1998 to July 2007. PVT was scorded according to the operative findings and Yerdel grading of PVT. No prisoners were used as donors for this study. Results Two hundred fifty-three patients were diagnosed with PVT (10.09%): there were 104 grade I; 114, grade II; 29, grade III; and 6, grade IV PVT. Sex and previous splenectomy increased the risk for PVT. In grade I and II cases, we performed simple thrombectomy, eversion thrombectomy, or improved eversion thrombectomy (IET, innovated by our center), producing smooth postoperative recoveries with a 0% in-hospitality mortality. In grade III cases, 18 underwent successful IET. Of 11 subjects who had eversion thrombectomy, six failed, and the distal superior mesentery vein or dilated splanchnic collateral tributary had to be used as the inflow vessel in four patients, and portal vein arterialization were performed in the other two patients, all of whom experienced a smooth postoperative recovery except one who died of hepatic failure and pulmonary infection 2 weeks after the operation. The in-hospitality mortality was 3.45%. In grade IV cases, three underwent successful IET, but another three cases failed, with two of them requiring a renal vein as the inflow vessel, and other one undergoing portocaval hemitransposition, with one postoperative death due to hepatic failure and another of cancer recurrence, an in-hospitality mortality rate of 33.33%. The transfusion requirement among PVT patients was significantly higher than that in non-PVT patients (9.32 ± 3.12 U vs 6.02 ± 2.40 U; P < .01). Blood loss in PVT patients who underwent the IET technique was significantly lower than that for an eversion thrombectomy (2800.36 ± 930.52 mL vs 5700.21 ± 162.50 mL P < .05). The overall actuarial 1-year survival rate in PVT patients was similar to the controls (86.56% vs 89.40%; P > .05). Conclusion OLT was successfully performed for PVT patients. The grade of PVT decided the surgical strategy. Similar 1-year survival rates were attained between PVT patients and controls undergoing OLT.