This monogenic disease compendium includes primary immunodeficiencies, autoinflammatory disorders, and defects in hematopoiesis, each with known causal genetic mutations affecting major molecular and cellular networks and functions of the innate [e.g., NOD-, LRR- and pyrin domain-containing protein 3 (NLRP3)] and adaptive [e.g., signal transducer and activator of transcription 1 (STAT1)] immune systems. This evidence concerns the gene NLRP3 and inborn error of immunity.