We report an 11-month-old boy of French origin who had chronic, infantile, neurological, cutaneous, and articular syndrome with a particularly severe joint involvement with early onset. The diagnosis was based on the association of neurologic, cutaneous (urticarial skin eruption), and articular manifestations accompanied by recurrent bouts of fever. No mutation of the CIAS1 gene could be identified. Skeletal involvement was particularly severe, leading to considerable limitation of motion.