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Introduction and Objectives: Congenital Portosystemic Shunts (CPSS) are rare vascular malformations that involve communication between the portal and the systemic venous system. Patients with this condition may be asymptomatic or present with severe complications such as hepatic encephalopathy (HE), hepatopulmonary syndrome (HPS), pulmonary arterial hypertension (PAH), or liver nodules (LN). This study aimed to share our experience in the diagnosis and treatment of patients with CPSS. Materials and Methods: This is an observational, retrospective study including patients diagnosed with CPSS between 2011 and 2022 in our hospital. Results: We present nine children between three months and sixteen years old at the time of diagnosis, which was incidental in four patients and due to CPSS complications in five patients: two presented HE, one HPS and two with LN (one adenoma and one focal nodular hyperplasia). According to the Bicetre classification, four cases were type I, three were type II and two were type IV. Six patients had CPSS-related congenital cardiopathies, one had polysplenia, and another patient had severe scoliosis. Two patients had genetic syndromes: Down Syndrome and Turner Syndrome. We obtained an angioCT or angioMRI in all cases; eight patients also underwent an interventionist study. Four patients underwent shunt closure; one patient was by interventionist radiology and the other three were by conventional surgery, as closure by interventionist radiology was not feasible. Both HPS and HE resolved after closure. Two of the other six patients died of cardiac complications, and none of the other patients have presented CPSS complications to date and are under evaluation for treatment strategies. Conclusions: As CPSS is a rare condition, it is advisable to consider a high diagnostic suspicion, mainly in patients with cardiovascular malformations and/or hyperammonemia of undetermined cause. Abdominal Doppler ultrasound should be considered as a baseline study. Given the complexity of the condition, a multidisciplinary approach is recommended.