Granular cell tumor is a rare cause of hepatic dysfunction. We report here on a patient who underwent liver transplantation for this ailment. In our literature review, the common bile duct was most commonly involved (56%). A wide variety of therapies were advanced for this type of lesion, spanning three decades of care. Twenty-eight patients (49%) had no follow-up reported, and another 2 (3%) were found at autopsy. Sixteen patients (28%) were followed more than 1 year, with 72% followed less than 1 year if at all. We present the first case of a granular cell tumor being treated with liver transplantation. Although adequate early excisional surgery should obviate the need for transplantation in these cases, widely disparate therapy and poor follow-up may mask generally inadequate therapy for this lesion. The authors recommend thorough excision and long-term follow-up for patients with this entity to avoid secondary biliary cirrhosis and to eliminate the preventable need for transplantation.