AbstractHyperimmunoglobulin D syndrome (HIDS) is a rare autoinflammatory disorder with autosomal recessive inheritance. It is caused by specific mutations in the mevalonate kinase gene (MVK). No treatment specific to HIDS has been approved to date; however, nonsteroidal anti‐inflammatory drugs, steroids, colchicine, tumor necrosis factor‐α inhibitors, and anti‐interleukin‐1 treatments are used, based on case reports and observational studies. Herein, we report a case with recurrent fever and arthritis attacks who did not respond to anakinra and was successfully treated with canakinumab. Long‐term remission was achieved without any side effects with 300 mg canakinumab treatment every 4 weeks for 5 years.