PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) syndrome is a relatively not wellrecognized periodic fever syndrome characterized by recurrent febrile episodes persisting 4-6 days in children. Fever is accompanied by aphtous stomatitis, pharyngitis, and cervical adenitis. There are several clinical similarities between familial Mediterranean fever (FMF) and PFAPA syndrome. For accurate diagnosis, increased awareness of this syndrome is essential. We describe a three-year-old girl with PFAPA who used colchicine prophylaxis with the presumptive diagnosis of FMF. At the follow-up, she was diagnosed as having PFAPA syndrome. The interval between the febrile episodes was increased from four weeks to 12 weeks after colchicine therapy. Since there are many similarities between the FMF and PFAPA syndrome, colchicine use can be offered as an effective approach for children with PFAPA

Keywords: PFAPA, colchicine, periodic fever, FMF