We present a seminal case of NOMID in a Chinese neonate, notable for several key aspects: it is the earliest genetically confirmed case in China, it harbors a rare NLRP3 (p.Gly755Arg) mutation, and it demonstrates the profound efficacy of very early intervention with canakinumab, initiated at 48 days of life, in achieving clinical and biochemical remission without emerging serious complications during the 13-month follow-up. Here, NLRP3 is linked to CINCA syndrome.