A 9-year old boy suffered from fever 20 days. He lived in rural area near farms rearing cows, goats, sheep. He had fever for 20 days with mild respiratory symptom, hepatosplenomegaly and agranulocytosis. Coxiella burnetii infection was confirmed by the blood culture and demonstration of the specific antibodies in the indirect fluorescent antibody test. Four weeks later, agranulocytosis was improved by long-term teratment of tetracylin and chloramphenicol. We repotr a case of Q fever with agranulocytosis with a brief review of literatires.