Portal vein thrombosis (PVT) is a poorly described complication of inflammatory bowel disease (IBD). We sought to better characterize presentations, compare treatments, and assess outcomes in IBD-related PVT. We conducted a retrospective investigation of IBD-related PVT at our institution. Multivariable Cox proportional hazards modeling was used to estimate adjusted hazard ratios across treatments. Sixty-three patients with IBD-related PVT (26 with Crohn disease, 37 with ulcerative colitis) were followed for a median 21 months (interquartile ratio [IQR] = 9-52). Major risk factors included intra-abdominal surgery (60%), IBD flare (33%), and intra-abdominal infection (13%). Primary hematologic thrombophilias were rare and did not impact management. Presentations were generally nonspecific, and diagnosis was incidental. Ninety-two percent of patients (58/63) received anticoagulation (AC), including 23 who received direct oral anticoagulants (DOACs), 22 who received warfarin, and 13 who received enoxaparin. All anticoagulated patients started AC within 3 days of diagnosis. Complete radiographic resolution (CRR) of PVT occurred in 71% of patients. We found that DOACs were associated with higher CRR rates (22/23; 96%) relative to warfarin (12/22; 55%): the hazard ratio of DOACs to warfarin was 4.04 (1.83-8.93; P = 0.0006)). Patients receiving DOACs required shorter courses of AC (median 3.9 months; IQR = 2.7-6.1) than those receiving warfarin (median 8.5 months; IQR = 3.9-NA; P = 0.0190). Incidence of gut ischemia (n = 3), symptomatic portal hypertension (n = 3), major bleeding (n = 4), and death (n = 2) were rare, and no patients receiving DOACs experienced these adverse outcomes. We show that early and aggressive use of AC can lead to excellent outcomes in IBD-associated PVT and that DOACs are associated with particularly favorable outcomes in this setting.