The treatment depends on clinical features and the location of the biliary abnormality. When the disease is localized to one hepatic lobe, hepatectomy relieves symptoms and appears to remove the risk of malignancy. Good evidence suggests that malignancy complicates<unk>  in roughly 7% of cases. Antibiotics are used to treat the inflammation of the bile duct, and ursodeoxycholic acid is used for hepatolithiasis. Ursodiol is given to treat cholelithiasis. In diffuse cases of<unk> , treatment options include conservative or endoscopic therapy, internal biliary bypass procedures, and liver transplantation in carefully selected cases. Surgical resection has been used successfully in patients with monolobar disease. An orthotopic liver transplant is another option, used only when antibiotics have no effect, in combination with recurring cholangitis. With a liver transplant, cholangiocarcinoma is usually avoided in the long run. Family studies are necessary to determine if<unk>  is due to inheritable causes. Regular follow-ups, including ultrasounds and liver biopsies, are performed.
Caroli disease
Haff disease
Darier's disease
Lev's disease
Machado–Joseph disease
Kyrle disease
Tornwaldt's disease
Chandler's disease