Familial Mediterranean fever (FMF) is recognized as an autoinflammatory disease with repeated serositis as the main symptom. FMF is a genetic disease that is known to occur in the Arab world, Turkey, and Armenia, all of which are located in the Mediterranean coastal region. FMF is rare in Japan, and the estimated total number of patients is about 300 cases. We report a pediatric patient who developed a fever of more than 38 degrees Celsius after undergoing dental treatment with physical restraint and who received dental treatment under anesthetic management at our university hospital. The pediatric patient (5 years old) had been diagnosed as a typical case of FMF based on a genetic examination. Oral colchicine was administered to prevent fever. Dental treatment under monitored anesthesia care was requested by our pediatric dentist to prevent postoperative complications. The patient had no medical history other than FMF and was classified as ASA-PS 1. Intensive dental treatment was performed under the continuous infusion of propofol (10 mg/kg/h) and the intravenous injection of acetaminophen (255 mg) to prevent fever at the end of the anesthesia. The patient was observed by the pediatrician during the postoperative period, and no post-operative complications, such as fever, occurred.
