NALP3, a member of Nod-like receptors, has a crucial role in inflammation and immunity and may be a proximal sensor of cellular stress and danger signals. NALP3 forms a caspase-1 activating molecular complex termed the inflammasome. The inflammasome allows activation of IL-1β, the key player of the inflammation and fever. NALP3 gene encodes a pyrin like protein which contains a pyrin domain, a nucleotide binding site (NBS) domain, and a leucine rich repeat (LRR) motif. NALP3 protein interacts with apoptosis associated speck like protein containing a CARD and may function as an inducer of apoptosis and an activator of NF-κB signaling. Defects in NALP3 have been associated with familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), chronic infantile neurological cutaneous and articular (CINCA) syndrome, and neonatal-onset multisystem inflammatory disease (NOMID).