The association of hepatolithiasis (HL) and peripheral cholangiocarcinoma (PCC) has been well recognized. However, information concerning the impact of hepatolithiasis on patients with peripheral cholangiocarcinoma is sparse and therefore difficult to assess. A total of 162 consecutive patients with histologically proven peripheral cholangiocarcinoma were treated surgically at Chang-Gung Memorial Hospital between 1977 and 1994. Among them, 106 patients (65.4%) had associated hepatolithiasis (PCC + HL group), and the remaining 56 patients (34.6%) did not (the PCC − HL group). The differences in demographics, symptomatology, laboratory data, tumor staging, histological pattern, resectability rates, and long-term survival of these two groups were compared. The male to female ratio was 0.7 in the PCC + HL group and 1.3 in the PCC − HL group (P < 0.05). Two thirds of the PCC + HL group presented with acute cholangitis, whereas two thirds of the PCC − HL group presented with hepatomegaly (P < 0.01). Those patients in the PCC + HL group were in earlier stages than those of the PCC − HL group at the time of the initial diagnosis (P < 0.05). The resectability rate for the PCC + HL group was 31.1% and for the PCC − HL group, 26.8% (P > 0.05). Surgical mortality rates were 3.8% in the PCC + HL group and 3.6% in the PCC − HL group (P > 0.05). The morbidity rate was much higher in the PCC + HL group than in the PCC − HL group (P < 0.01). The 1-, 3-, and 5-year survival rates were 35.5%, 20.5%, and 16.5% in the PCC + HL group and 27.2%, 8.8%, and 7.8% in the PCC − HL group (P > 0.05). In conclusion, the presence of hepatolithiasis hindered an exact diagnosis of underlying cholangiocarcinoma preoperatively, precipitated biliary sepsis which affected resectability, and increased postoperative morbidity. Hepatolithiasis per se, however, did not influence the long-term survival.