Periodic fever syndrome (PFAPA syndrome) is characterized by periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. Typical febrile episodes last for 5 days and recur every 28 days. Other characteristic features of this syndrome are usually relatively well being of the children during episodes and completely well being between the intervals of these episodes as well as normal growth and development. The cause of PFAPA syndrome is unknown. Immundisregulation or infections are thought to play role in etiopathogenesis. There is no specific diagnostic test for PFAPA syndrome. During a febrile episode, mild leukocytosis and elevated erythrocyte sedimentation rate commonly occur. The differential diagnosis of PFAPA syndrome includes cyclic neutropenia, hyperimmunoglobulinemia D syndrome, Familial Mediterranean Fever, Hibernian fever and Behcet’s disease. In the treatment of this syndrome, usage of prednisone during febrile episodes and cimetidine for the prophylactic therapy are recommended. Tonsillectomy is preferred in some selected cases. In this review general characteristics of this newly described syndrome of childhood are highlighted.