ABSTRACT
Rare autosomal dominant disorders associated with various tumors and cysts, and several kinds of pancreatic lesions have been described in patients with von Hippel- Lindau (VHL) disease, but there have been few studies concerned with the variations of the pancreatic duct. We experienced a case of pancreatic dual Wirsung ducts in a patient with VHL disease. Computed tomography scans showed that multiple cysts had nearly completely replaced the pancreas, there was a solid tumor on the uncinate process of the pancreas and a left renal mass suggested renal cell carcinoma. ERCP shows that the Santorini duct and Wirsung duct were fused in a normal fashion, and another Wirsung duct had shrub-like branches that are commonly seen in pancreatic divisum.