Genetic sequencing confirmed the diagnosis of PAPA syndrome, with the pathogenic mutation c.688 G > <i>A</i> (p. Ala230Thr) in the PSTPIP1 gene present in the patient and in the mother.<h4>Conclusions</h4>This case illustrates that in children with recurrent/recalcitrant sterile recurrent pyogenic arthritis/osteomyelitis, the possibility of an underlying immunological condition should be considered. The gene discussed is PSTPIP1; the disease is arthritic joint disease.