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Context Von Hippel-Lindau disease is a genetic disorder characterized by neoplasms with multiple organ involvement, the pancreas being involved in about half of the cases. Conservative treatment is indicated because the disease is usually asymptomatic with long-term follow-up. Case report We herein present the case of a 64-year-old man with von Hippel-Lindau disease who presented with obstructive jaundice which resulted as being caused by a fibro-cystic pancreatic nodule. In addition, we reviewed the literature concerning pancreatic involvement in von Hippel-Lindau disease with emphasis on their presentation, type of lesions and appropriate management, especially in cases with obstructive jaundice. Conclusion Conservative management is advocated in the majority of VHL disease patients with pancreatic involvement, but surgery is sometimes required, especially when patients are symptomatic (obstructive jaundice, upper gastrointestinal bleed).