A 55-year-old male presented with a recurrent fever of over 38 degrees C, occurring at irregular intervals 1-6 times a month with chest, back or abdominal pain. After admission to our hospital, we found the following characteristics: 1) the febrile attacks were accompanied by obvious inflammatory findings and pleuritis or peritonitis; 2) the patient's elder sister had a similar periodic fever; and 3) there were no apparent causative factors responsible for his symptoms. Therefore, we diagnosed this as a case compatible with familial Mediterranean fever. The febrile attacks have been completely suppressed by daily colchicine. This is the seventh case of familial Mediterranean fever reported in Japan.