Budd-Chiari syndrome (BCS) is a rare hepatic disease caused by occlusion of the hepatic venous outflow at any level from the small hepatic veins to the atriocava junction. BCS could have serious consequences if not treated promptly. The appropriate therapeutic strategy can be offered to change the natural course of the disease. The present case reports a young man with BCS who successfully received the hybrid treatment combined with endo-vascular intervention and mesocaval shunt by step. The 11-year follow-up showed that the patient was free of clinical symptoms, and computed tomography and ultrasonography confirmed the patency of the stent and shunt. Although BCS therapy methods are well established, the patient often needs to be treated repeatedly because of the high risk of recurrence. Step therapeutic strategy to alleviate portal and inferior vena cava hypertension of BCS patients are respected. The combination of endo-vascular intervention and mesocaval shunt was effective in our patient, and both stent and shunt have satisfactory long-term patency.