We encountered 4 cases of hepatic peribiliary cysts (HPBC) in our hospital. Two were asymptomatic, one was complicated by a choledocholithiasis, and one by cholangitis and sepsis. Based on a review of the 38 cases of HPBC clinically diagnosed in Japan, the main problem associated with this disease seemed incorrect preoperative diagnosis leading to an unnecessary hepatectomy. Another problem was concomitant cholangitis, which tended to recur and in some cases needed aggressive treatment using drainage.