Abstract: Objective To compare the clinicopathological features of biliary cystadenoma (BCA) with those of biliary cystadenocarcinoma (BCAC), and explore the related factors of prognosis. Methods Clinical data about 39 patients with clinicopathologically confirmed BCA or BCAC in Chinese PLA General Hospital and Anshan Central Hospital from January 2005 to December 2014 were collected and reviewed, their clinical features, laboratory examination, radiologic findings, surgical treatment and prognosis were compared. Results Thirty-nine patients with pathologically diagnosed BCT (BCA, n=23; BCAC, n=16) were included (7 males and 32 females), with an average age of (47.3±15.5) years (7-77 years). The postoperative pathological examination confirmed 23 cases of BCA and 16 cases of BCAC. BCAC was significantly more frequently associated with liver dysfunction compared with BCA (P＜ 0.05). Though the presence of mural nodule was significantly more frequent in the BCAC group than in the BCA group (10/16 vs 7/23, P=0.047), the radiologic findings in other aspects such as capsule wall integrity, septa, calcification, wall or septa enhancement showed no significant differences (AllP＞ 0.05). All patients underwent surgical resection while 32 patients had complete resection and 7 patients had incomplete resection. Twenty-nine patients were followed up with a median of 40 months (3-124 moths), and the median survival time of BCAC group was 41 months. Conclusion Preoperative differential diagnosis in BCA and BCAC still remains difficulties, and accurate diagnosis should be based on postoperative pathology. BCT occurs predominantly in middle-age women, and the prognosis of BCAC is poor.