Fourty-four of 389 patients with VHL (between 1988-1999) screened positive for PNETs either by pathologic analysis of tissue specimens or by characteristic radiographic appearance on CT and MRI. VHL gene germline mutations were determined by quantitative Southern blotting to detect deletions of the gene and gene rearrangements as well as complete gene sequencing. One patient (15), a 26 year old female, was found to harbour the p.Val181Glyfs*20 (c.540_543delCGTC) VHL gene mutation, stated in the paper as c.753_756delCGTC according to an older transcript reference. She presented at 21Y with a single radiologic pancreatic neuroendocrine tumor. No family information was present.