Two granular cell tumors of the biliary tree are described, one in a 37-year-old black woman with obstructive jaundice and the other in a 26-year-old white woman with abdominal pain. These are rare soft tissue tumors that have an excellent prognosis when surgically excised. Almost all reported cases have been in young women, the majority of whom have been black. Because granular cell tumors of the biliary tract are uniformly resectable and curable, they should be considered and distinguished from cholangiocarcinoma or localized sclerosing cholangitis, particularly if the patient is young, female, and black.