Objective:To investigate the feasibility and outcome of partial hepatectomy and fenestration combined with renal cyst decortication for treatment of autosomal dominant polycystic liver disease (APLD) associated with autosomal dominant polycystic kidney disease (APKD).Methods: From July 2007 to Oct.2007,three patients with APLD associated with APKD were treated by partial hepatectomy and fenestration combined with renal cyst decortication in our hospital.Their preoperative symptoms,operation procedure,operation time,complications and the clinical outcomes were retrospectively analyzed.Results: The 3 patients were successfully treated and discharged.The operation time periods of the 3 patients were 475,402,and 430 minutes.Pleural effusion and ascites occurred in all the 3 patients and disappeared after puncture and drainage.Follow-up was conducted 5,6,and 9 mouths later; the symptoms disappeared in all patients and the renal function became normal.The blood pressure of 2 patients recovered to normal level after operation.Conclusion: Partial hepatectomy and fenestration combined with renal cyst decortication is a safe and acceptable procedure for treatment APLD associated with APKD.The complication rate is comparatively more and the long-term outcomes need to be evaluated further.