PFAPA syndrome is the most common periodic fever syndrome in our geographic zone. It usually develops in children under 5 years of age and is classified as an autoinflammatory disease. PFAPA syndrome is characterised by episodes of high fever (>39°C) accompanied by aphthous stomatitis, pharyngitis, and cervical lymphadenopathy, which occur cyclically at 25–35-day intervals and last between 3 and 6 days. Patients experience no symptoms between these episodes. The disease resolves with age. The cause of PFAPA and the predisposing factors remain unknown. The paper presents a clinical case of a 2-year-old girl diagnosed in the Department of Paediatric Pulmonology and Rheumatology of the Medical University of Lublin due to recurrent fever episodes. After exclusion of other causes, PFAPA syndrome was diagnosed. Corticosteroid treatment was used and good therapeutic response was achieved. The paper also presents a literature review on the current diagnosis and treatment modalities in PFAPA.