Context Cystic lesions of the pancreas are a common finding in patients with von Hippel-Lindau disease (vHL). However, only twelve cases of pancreatic cystic lesions causing obstructive jaundice have been reported in vHL patients. Case report Herein we report the rare case of a massive serous cystadenoma of the pancreatic head causing obstructive jaundice in a fifty-one-year-old female with vHL. Preoperative biliary decompression was achieved via ERCP and stent placement. Since the whole pancreas showed cystic degenerations, a total pancreatectomy was performed. Final histopathology showed diffuse compression of the distal intrapancreatic common bile duct but no evidence of malignancy. Conclusion To our knowledge this is only the second case report of a total pancreatectomy performed in a patient with vHL for biliary obstruction caused by a serous cystadenoma of the pancreas. Surgical intervention in this rare scenario should be recommended, because of the inability to rule out malignancy and the necessity to address the biliary obstruction.