Hemorrhage at presentation in primary CNS lymphoma is exceedingly rare. We describe a patient with primary CNS lymphoma who presented with seizures and was found to have an intracerebral hemorrhage which prompted a cerebral angiogram. Ultimately pathologic evaluation of the lesion revealed a highly cellular B-cell lymphoma with marked hypervascularity and intense expression of vascular endothelial growth factor (VEGF). A review of four other recent cases of primary CNS lymphoma at our institution revealed less-intense VEGF immunoreactivity. This is the first report of VEGF expression in primary CNS lymphoma. The potential significance of VEGF expression with respect to the biology of this tumor is discussed.