PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, cervical adenopathy) is the most common periodic fever syndrome occurring in children. Exacerbation period is characterised by episodes of high fever with pharyngitis, cervical lymphadenopathy and increased inflammatory markers. It is believed that the PFAPA syndrome does not increase sepsis markers. In the paper, we present a clinical case of a 6-year-old girl who was presented with recurrent fever episodes. After exclusion of other causes, PFAPA syndrome was diagnosed. Recurrences of fever were accompanied by an elevated level of procalcitonin and C-reactive protein. Corticosteroid treatment caused a permanent remission of the disease symptoms. Discussion is focused on increased inflammatory markers and the differentiation of the syndrome with generalised bacterial processes. Widening the knowledge of the syndrome among general practitioners and paediatrics is crucial for early diagnosis and implementation of proper treatment.