Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder which is associated with multiple tumors and cysts in the central nervous system (CNS) and other visceral organs. The most commonly seen tumors are hemangioblastoma in the CNS and retina, pheochromocytoma in the adrenal gland, renal cell carcinoma and pancreatic neuroendocrine tumors. Here we report a 38 year old lady who presented with headache, vomiting, vertigo and ataxia and non specific abdominal pain, who was diagnosed to

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... ve von Hippel-Lindau disease. The pathophysiology involves the inactivation of the VHL tumor suppressor gene present at 3p25-26 with results in loss of function of the VHL protein, and Elongin B, C complex resulting in a dysfunction of the ubquitination of hypoxia-inducible factor, which is a crucial step in the development of highly vascular tumors. There is no definitive treatment available till date. Management basically aims at early recognition and treatment of specific manifestations in order to decrease complications and improve the quality of life. Increasing knowledge about the molecular role of VHL proteins (pVHLs) has led to investigate the role of antiangiogenic drugs designed to reduce or prevent tumorogenesis in VHL disease.