CINCA syndrome is an autoinflammatory syndrome characterized by neonatal onset of urticarial rash, central nervous system lesions and arthropathy. Laboratory findings show leucocytosis, anemia, elevation of CRP levels and acceleration of ESR. The syndrome is associated with CIAS1 gene and its encoding protein cryopyrin. The CIAS1 gene is expressed in monocytes, polymorphonuclear cells and chondrocytes. Mutations of cyropyrin lead to the persistent production of IL-1beta and activation of NF-kappaB, followed by excessive inflammtory reactions. In spite of aggressive therapies, the inflammation persists and the lesions are progressive. Recently, there have been reports of clinical improvement using human recombinant IL-1 receptor antagonsist anakinra in the patients with CINCA syndrome and its related diseases, Muckele-Wells syndrome and familial cold-autoinflammatory syndrome, suggesting that IL-1beta plays an important role in the pathogensis of the inflammation associated with the CIAS1 gene mutations. No serious adverse reaction of anakinra has not been reported. Following the diagnosis of CINCA syndrome, anakinra therapy should be started as the first line of therapy before irreversible disabilities develop. Treatment with anakinra has just begun, therefore, it is necessary to carry out further investigations concerning the adverse effects of anakinra and long-term prognosis for CINCA syndrome treated with anakinra.