von Hippel Lindau syndrome presenting as obstructive jaundice with involvement of pancreas in two siblings.

Von Hippel Lindau disease is rare autosomal dominant disorder. In our patient's family, 4 out of 7 siblings had manifestations of the disease; 3 of those affected had pancreatic lesions with obstructive jaundice in 2 siblings caused by pancreatic cysts is reported for the first time.