Stenosis of the portal vein (PV) is a rare complication after liver transplantation (LT) in pediatric patients, and it adversely affects outcomes. We reviewed the safety and efficacy of percutaneous transhepatic balloon angioplasty (PTBA) as a treatment for post-LT late-onset PV stenosis (PVS). Three hundred eighteen patients between the ages of 0 and 21 years received an LT from 2001 to 2016 at this tertiary center. Twenty-one children were evaluated for PVS using percutaneous portal venography. Of the 21, 19 patients (7 female, 12 male) with a median age of 12 years (7-15 years) were diagnosed with PVS and received PTBA. Two patients were excluded: one did not have PVS, and one received shunt surgery. Median time between LT and PTBA was 83 months (interquartile range, 49-138). For patients in whom pressure could be accurately measured (n = 9), mean PV pressure gradient was 6.3 mm Hg (SD, 5.0) preprocedure and 0.9 mm Hg (SD, 1.2) postprocedure. Mean percentage improvement in gradient across the stenotic region was 86.2% (SD, 15.9%; P < 0.01). At 12-month postprocedure, there was a mean improvement (pre-PTBA vs post-PTBA means) in bilirubin by 28.2% (0.6 mg/dL vs 0.4 mg/dL, P = 0.07), aspartate aminotransferase by 31.2% (116.3 IU/L vs 28.1 IU/L, P = 0.04), alanine aminotransferase by 40.7% (140.3 IU/L vs 28.6 IU/L, P = 0.07), γ-glutamyltransferase by 29.0% (337.2 IU/L vs 38.0 IU/L, P = 0.06) and platelets by 62.1% (128.3 vs 191.1 × 10/L, P = 0.03). The PV patency was successfully maintained in 18 of 19 patients for a median of 16 months (interquartile range, 5-35). One patient received a successful repeat procedure for restenosis at 6 weeks. Angioplasty for PVS after pediatric LT is a safe and effective treatment with good patency and improved clinical outcomes. Longer follow-up studies are required.