Background: Supratentorial ventricular hemangioblastoma (HB) associated with Von Hippel Lindau (VHL) are extremely rare. Due to their vascularity and location, their management can be difficult. Methods: A 35 year old female with VHL, has been followed for 25 years with multiple intracranial and spinal tumours. Surgical removal was carried out on one large cystic and solid posterior fossa lesion. In addition, she underwent adrenalectomy for pheochromocytoma. There were no pancreatic or renal lesions. On serial follow up for years, a left frontal ventricular lesion showed increasing size with clinical signs of increased ICP and marked hydrocephalus, requiring shunting procedures, which were carried out 11 years ago. She has been clinically stable since. Results: Hemangioblastomas of the CNS are rare and account for 2% of primary CNS tumours. Supratentorial location is estimated at 4% for sporadic and 13% for HB associated with VHL. The lateral ventricular location is extremely rare. Review of the literature revealed a total of 9 cases of supratentorial ventricular location. The majority of the lesions are associated with VHL and they are solid and vascular lesions. In our cases there was a cystic component. Conclusions: If removal is contemplated, angiography with possible preoperative embolization may be required.