von Hippel-Lindau disease is an autosomal dominant inherited disease with a wide spectrum of manifestation which causes benign and malignant tumors in multiple systems. We present a case of von Hippel-Lindau disease (VHL) who initially presented with retinal hemangioblastomas, but after 10 years had tumors in multiple organs,and few benign conditions. This signifies the importance of annual screening for detection and monitoring of conditions which can lead to timely surgical intervention and improve morbidity and mortality for patients. Radiological imaging plays a crucial role in diagnosing, monitoring, and screening for various manifestations and along with newer surgical techniques have contributed to significant reduction in morbidity and mortality of VHL patients. It is important to have a multidisciplinary team approach in screening for VHL disease