PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis) syndrome has characteristic features of an early onset, periodicity (interval <4 weeks), and a brisk rising high fever (>39 celsius) of 3 to 6 days' duration without signs of infection. An 18- year-old male was admitted for high fever, sore throat, headache, and abdominal pain. He had suffered from periodic fevers associated with pharyngitis and cervical adenitis for 14 years without any evidence of infection. The fever recurred approximately every 4 weeks lasting 4 to 7 days with intervening asymptomatic periods. Physical examination was unremarkable except for slightly injected tonsils. Most laboratory tests including CBC were normal except for elevation of C- reactive protein level (3.47 mg/dL). Bacteriologic and radiologic studies were negative. The fever was spontaneously subsided in 6 days. To our knowledge, this is the first case report of PFAPA syndrome in Korea.