Based on the findings of a patient with recurrent obstructive jaundice due to papillomatosis of the distal bile duct, we herein describe the diagnostic difficulties and therapeutic options in this very rare disease. Endoscopic retrograde cholangiopancreatography and, in particular, cholangioscopy are the imaging procedures of choice if biliary papillomatosis is suspected. Due to the tendency of such patients to demonstrate malignant transformation and develop biliary cirrhosis with septic complications, an early and radical surgical resection is recommended in rare cases of localized papillomatosis. This approach may offer the only chance of a cure although the potential risk of multifocal recurrence cannot be ruled out. When considering a radical resection, intraoperative cholangioscopy is strongly recommended to confirm any localized papillomatosis and rule out any diffuse papillomatosis of the entire biliary tract.