AbstractObjectiveNon‐cirrhotic portal vein cavernoma (PVC) is a cause of portal hypertension (PH) frequently affecting women of childbearing age. Cavernous transformation of the portal vein is frequently associated with prothrombotic disorders and often entails multiple hemodynamic changes, porto‐collateral shunt development and thrombopenia, all of which can affect the course of pregnancy. Our aim was to evaluate the risk of PH‐related complications and pregnancy outcomes in patients with PVC.MethodsRetrospective case series study of patients with PVC undergoing pregnancy in a tertiary care hospital.ResultsEight pregnancies fulfilled the eligibility criteria. All patients had a predisposing factor for PVC. One episode of variceal bleeding was reported at week 28. Six cesarean sections were scheduled to avoid labor while two urgent surgeries were indicated due to fetal distress and intrauterine growth restriction (IUGR). In all but one case, anticoagulation was prescribed after delivery. No hemorrhagic or thrombotic complications were reported. There were four cases of IUGR with no case of miscarriage or stillbirth.ConclusionPregnancy in patients with PVC has an overall favorable outcome albeit a higher risk of PH‐derived complications, and IUGR may be expected. Hence, PVC must not be considered a contraindication for pregnancy although larger prospective studies are necessary.