Fourth ventricular hemangioblastoma associated with pheochromocytoma and renal medullary fibroma

Intraventricular hemangioblastomas are exceptionally rare. Of the cases described in the literature, very few were associated with von Hippel-Lindau disease. We present a highly unusual case of a fourth ventricular hemangioblastoma associated with a pheochromocytoma and a renal medullary fibroma. This may represent a forme fruste of the von Hippel-Lindau complex. A workup for papilledema resulted in the discovery of this rare finding.