Biliary tact carcinoids are extremely rare: only ten cases have been reported up to now. The Authors describe a successfully treated carcinoid tumour of the proximal bile duct and review the literature about these rare malignancies. Despite extensive preoperative work-up, including ultrasound, CT scan and ERCP, a definite diagnosis is hardly possible prior to histologic examination of the operative or necropsy specimen. Due to the slow-growing nature and the non-aggressive behaviour of these malignancies, surgical resection followed by biliodigestive anastomosis should be the treatment of choice.