Published online Oct 16, 2013. doi: 10.4253/wjge.v5.i10.519
Revised: August 25, 2013
Accepted: September 4, 2013
Published online: October 16, 2013
Core tip: This is a case of a rare clinical entity, Von Hippel-Lindau disease, with an unusual presentation. The patient had only pancreatic cysts without more common manifestations, particularly hemangioblastomas and malignancy. The imaging methods used in this case were important for the diagnosis, particularly endosonography, which showed the honeycomb appearance of the pancreatic serous cystadenomas. This case should alert endoscopists to the possible occurrence of this hereditary disease in the presence of multiple pancreatic cysts without other manifestations or family history.