Q fever has been generally regarded as an acute, self-limited respiratory illness. In the case here presented, the patient, a man aged 40, had fever, extrapyramidal neuropathy, and granulomatous hepatitis which pursued a chronic course without pulmonary involvement. More than 30 cases of Q fever hepatitis have been reported. This is, however, the first case of central nervous system disease with onset marked by extrapyramidal neurological signs. The spectrum of reported clinical manifestations caused by infection with Coxiella burnetii includes meningitis, encephalitis, pericarditis, endocarditis, pleuritis, pneumonia, arteritis, pancreatitis, orchitis, and epididymitis. Since Q fever is prevalent throughout the United States, an awareness of its diverse clinical manifestations is essential. Q fever, unlike many other diseases that mimic it, is effectively treated with antibiotics if they are used early.