Neuroendocrine Tumor of the Common Bile Duct Associated With von Hippel-Lindau Disease

We report a case of a common bile duct neuroendocrine tumor discovered in a patient with von Hippel-Lindau disease to emphasize the importance of recognizing this unusual diagnosis. This case illustrates the importance of endoscopic evaluation and the potential diagnostic pitfalls which may impact its appropriate management: the anatomic proximity of more common von Hippel-Lindau disease–related tumors, pathologic evaluation, and staging. Therefore, awareness of this rare diagnosis is important for appropriate treatment.