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A 41-yr-old patient with a history of von Hippel-Lindau (VHL) disease with previously removed bilateral pheochromocytomas and renal cell carcinoma presented with progressive obstructive jaundice due to multiple lesions in the pancreas. The pancreatectomy specimen showed a range of endocrine lesions including islet hyperplasia, nesidioblastosis, microadenomas, and endocrine carcinoma. In addition, some of the non-tumorous islets displayed peliosis. The endocrine carcinoma showed a biphasic pattern composed of typical endocrine cells and oncocytes. The oncocytic component showed widespread lymphovascular invasion and lymph node metastasis. Immunohistochemistry and electron microscopy confirmed that the oncocytic cells were endocrine. Focal areas contained cells with foamy cytoplasm, a feature that is associated with pancreatic endocrine tumors in VHL.This case expands the spectrum of lesions seen in the pancreas of VHL patients. There is some overlap with lesions encountered in multiple endocrine neoplasia type I. In addition, the endocrine lesions were composed of two main cell types (typical and oncocytic cells) with the oncocytic component invading lymphatic channels and spreading to regional lymph nodes.