We report the clinical and radiological central nervous system manifestations of a 27-year-old man with Q fever who subsequently developed acute disseminated encephalomyelitis and showed a significant response to steroids. The patient presented with headache and fever and quickly progressed to develop acute respiratory failure and hepatitis. A prompt evaluation revealed positive serology for Q fever and doxycycline was initiated. Approximately 1 week into his illness he was noted to be profoundly weak. Neuroimaging with magnetic resonance imaging (MRI) revealed diffuse white matter T2/FLAIR hyperintensities, with evidence of restricted diffusion. He was given high-dose steroids for a presumed diagnosis of acute disseminated encephalomyelitis (ADEM) and within days he had both clinical and MRI improvement. In addition to well-described meningitis and encephalitis, Q fever may also be associated with diffuse CNS lesions that may be demyelinating inflammatory in pathophysiology, and therefore responsive to high-dose steroids.