In this pilot study, we aimed to investigate: (1) an association of genetic polymorphisms of NLRP3-inflammasome signaling with AOSD susceptibility using a MassArray and subsequent replication; (2) the associations of genotypes and alleles with clinical manifestations and disease outcome; and (3) the functional association of the involved gene polymorphisms in AOSD. The gene discussed is NLRP3; the disease is adult-onset Still disease.