BackgroundNeonatal portal vein thrombosis (PVT) is currently more commonly encountered as a result of advances in diagnostic tools and increase in invasive interventions.MethodsIn this study, 11 premature and 12 term infants diagnosed with PVT were retrospectively evaluated for clinical and laboratory characteristics, umbilical catheterization procedure, PVT location, risk factors, treatments, and long-term outcomes.ResultsMedian age of the patients at diagnosis was 10 days (range 3–90 days), and 69.6% of patients were girls. Of the 23 patients, 87% had left PVT and, 91.3% had at least one thrombosis risk factor, which was sepsis in 73.9% of patients, and presence of umbilical venous catheter in 87%. Totally, 59.1% of PVTs were completely resolved in a median follow-up of 7 months (1 month to 12 months), and 78.3% of these patients had no anticoagulant therapy (ACT). Partial thrombus resolution was achieved in 9 patients (40.9%). Five patients (%21) received ACT. Overall, 34.8% of patients had long-term complications. neonatal PVT is most commonly reported in the left portal vein and there is no evidence for the impact of ACT on reducing the short- or long-term complications. Well designed and larger studies are necessary to clarify this issue, which can facilitate developing appropriate management algorithms.ConclusionNeonatal PVT is most commonly reported in the left portal vein and there is no evidence for the impact of ACT on reducing the short- or long-term complications. Well designed and larger studies are necessary to clarify this issue, which can facilitate developing appropriate management algorithms.