A 3 ½-year-old previously healthy female experienced an episode of sudden unresponsiveness witnessed by her mother. Upon arrival to the local hospital, imaging studies of the still unresponsive child revealed severe bilateral "flash" pulmonary edema and diffuse anoxic brain injury. Aggressive resuscitative efforts were unsuccessful, and she was pronounced dead. External examination at autopsy was essentially unremarkable. Internal examination of the head revealed diffuse basilar subarachnoid blood originating from a collapsed, 2 cm irregular aneurysm arising from the junction of the vertebral and basilar arteries. Additionally, multiple calcified subpleural, parenchymal, and hilar nodal pulmonary granulomas were identified. The remaining internal examination, including that of the aorta and its major branches, was unremarkable. Histologic examination of the aneurysm revealed alternating mural attenuation and thickening, the latter resulting from prominent intimal proliferation with active fibroplasia. Most notably, numerous isolated and clustered multinucleated giant cells were seen, most prominently in areas of more intense inflammation. Specific immunolabeling and silver staining of the pulmonary granulomas revealed evidence of histoplasmosis, but both were negative for fungal elements in the aneurysm, as was ultrastructural examination. The cause of death is fatal subarachnoid hemorrhage due to rupture of a vertebrobasilar artery aneurysm caused by isolated intracranial giant cell arteritis.