AIM: To evaluate outcomes in patients with autosomal dominant polycyst liver disease (APLD) treated by combined hepatic resection and fenestration. A new classification was recommended to presume postoperative complications and long outcome of patients.METHODS: Twenty-one patients with APLD were treated by a combined hepatic resection and fenestration technique. All patients were reviewed retrospectively, and clinical symptoms, performance status and morbidity were recorded. A new classification of APLD is recommended here.RESULTS: All patients were discharged when free of symptoms. The mean follow-up time was 55.7 mo and three patients had a recurrence of symptoms at 81,68 and 43 mo after operation, respectively. The overall morbidity rate was 76.2%. Two patients with Type B-Ⅱand Type B- Ⅰ developed biliary leakage. Four patients had severe ascites, including three with Type B-Ⅲ and one with Type B-Ⅱ. Nine patients had pleural effusion,including one with Type A-Ⅰ; one with Type B-Ⅰ;five with Type B-Ⅱ; one with Type A-Ⅲ and one with Type B-Ⅲ. Three patients with Type B had recurrence of symptoms, while none with Type A had severe complications.CONCLUSION: Combined hepatic resection and fenestration is an acceptable procedure for treatment of APLD. According to our classification, postoperative complications and long outcome can be predicted before surgery.