The extremely rare, dominantly inherited PLCγ2-associated antibody deficiency and immune dysregulation (PLAID) and autoinflammation and PLCγ2-associated antibody deficiency and immune dysregulation (APLAID) may be included among those rare monogenic AIDs combining sterile inflammation and humoral immunodeficiency. The gene discussed is PLCG2; the disease is autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation.