Context Von Hippel Lindau disease is a rare autosomal dominantly inherited multisystem disorder characterized bydevelopment of benign and malignant tumors. The abdominal manifestation of the syndrome are protean. Magneticresonance plays an important role in identification of abdominal abnormalities and follow-up of lesions. Objective Todescribe magnetic resonance imaging findings and patterns of pancreatic and other principal abdominal manifestations in aseries of von Hippel-Lindau (VHL) disease patients and to review literature. Methods We retrospectively reviewedabdominal magnetic resonance studies performed in 23 patients (10 males, 13 females) diagnosed of VHL. Results In allexamined patients abdominal involvement was present. The pancreatic imaging findings detected were: unilocular cysticlesions (6/23: 26.1%); serous cystadenomas (11/23: 47.8%), including diffuse lesions (8/23: 34.8%); solid neuroendocrinetumors (8/23: 34.8%); cystic neuroendocrine tumors (1/23: 4.3%). The renal findings detected were: simple renal cysts(18/23: 78.3%); complex renal cysts (13/23: 56.5%), including benign lesions (10/23: 43.5%) and malignant lesions (3/23:13.0%); renal carcinomas (11/23: 47.8%) and 5 of these (45.5%) were multiple and bilateral. Five patients (21.7%)presented pheochromocytoma (4 of these were bilateral; 80.0%) and 1 patient (4.3%) presented cystadenoma of theepididymis. Conclusions In VHL disease patients, magnetic resonance imaging plays an essential role in the identification ofpancreatic and other abdominal lesions, in their follow-up, in the screening of asymptomatic gene carriers, and in their longtermsurveillance.