The patient is a 34-year-old patient with abdominal pain, gross hematuria with anxiety and worries about it from 5 months ago. The physician requested renal computed tomography (CT) without and then with contrast for rule out of renal stone. However, he found multiple lesions in kidneys. The laboratory tests were normal except hematuria. He was a candidate for surgery. The pathologist reported clear red cell renal cell carcinoma. He was referred to a radiologist for staging. Von Hippel – Lindau (VHL) disease is an inherited and rare disease that is characterized by a variety of benign and malignant lesions (1). It preval ence is 1 in 31,000 -53,000 (2,3). Previous studies shown 59 – 63% of patients have renal cysts and 24 - 45 % renal cell carcinoma (4), and in 75 % of cases ,the lesions are bilateral (4, 5). Involvement of pancreas includes simple cysts (50 – 91%), serous m icrocystic adenomas (12%) and adenocarcinoma (7%) (2, 4).