Portopulmonary hypertension (PoPH) is characterized by the combination of pulmonary arterial hypertension (PAH) and portal hypertension or portosystemic shunt. Although 1%-5% of children with portal hypertension develop PoPH, the prevalence in those with congenital portosystemic shunt reaches up to 15%. The prognosis of patients with PoPH is poor, with a 5-year survival rates of 35%-50%. Pediatric data on PoPH management remain limited and extrapolated from adult guidelines. In this study, we present the case of a child who underwent 2 separate surgeries aimed at restoring intrahepatic blood flow, each with distinct preoperative hemodynamic profiles, resulting in 2 different outcomes. The patient and her parents provided written informed consent for this report.