It has been known that intrahepatic biliary lithiasis (IHBL) is prevalent in East Asia including Japan, South Korea, Taiwan, Malaysia, Hong Kong, and Singapore. In contrast, the entity has drawn little attention in Europe and the United States where only scattered reports appear. IHBL can be placed in the category of the benign disease. Its distinctive clinical picture is an intractable course necessitating multiple surgical interventions because recurrence is usual, rather than exceptional. This is in distinct contrast to ordinal stones which originate in the gallbladder. Patients with IHBL do not rarely die of progressive hepatic damage resulting from longstanding obstructive jaundice, cholangitis, liver abscess, septicemia, and so forth.