ObjectiveTo summarize the treatment of a patient with congenital portosystemic shunt (CPS) complicated with hepatic focal nodular hyperplasia (FNH), and to explore the feasibility and safety of combined laparoscopy and interventional radiology therapy at the same time.MethodsThe clinicopathological data of a patient with CPS complicated with hepatic FNH who admitted to West China Hospital of Sichuan University in March 2019 was retrospectively analyzed.ResultsThe patient underwent laparoscopic liver nodule resection and digital subtraction angiography (DSA) guided jugular portal portosystemic shunt fistula embolization. The laparoscopic surgery operation time was 180 min and the intraoperative blood loss was 50 mL, and for interventional procedure was 230 min and 10 mL respectively. There were no complications after operation and the patient was successfully discharged on the 8th day after surgery. The patient was followed up for six months and in good condition.ConclusionsCPS patient should develop individualized treatment under the discussion of multidisciplinary cooperation group. The combination of laparoscopy and interventional technique can be minimally invasive and efficient to solve portal vein-avitary shunt fistula and benign hepatic nodules at the same time.