Caroli's disease epidemiology and demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

• The disease is uncommon, with about 180 cases reported in the literature.

• The disease affects about 1 in 1,000,000 people, with more reported cases of Caroli syndrome than of Caroli disease.

• Biliary cysts are more common in Japan and in young women.

• Females are more prone to Caroli disease than males.