The prevalence of CINCA syndrome, also known as neonatal-onset multisystem inflammatory disease (NOMID), is extremely rare. It is estimated to affect approximately 1 in every 1 million individuals worldwide. This chronic autoinflammatory disorder typically manifests in infancy and is characterized by persistent inflammation in multiple organs, including the skin, joints, and central nervous system. Due to its rarity, CINCA syndrome poses significant challenges in diagnosis and treatment. Early recognition and appropriate management are crucial to improve the quality of life for individuals living with this condition.