Hyperimmunoglobulin D and periodic fever syndrome (HIDS) is a rare, autoinflammatory condition caused by mutations in the mevalonate kinase gene. There is no standard treatment for HIDS, and randomized controlled trials are lacking. Corticosteroids, colchicine, nonsteroidal anti-inflammatory drugs, statins, and cyclosporine are of limited efficacy in controlling this condition. Recent case reports suggest that most patients respond to etanercept or anakinra. Interleukin 6 blockade in HIDS has not been described. We report the case of a 13-year-old girl with HIDS, who failed to respond to colchicine, corticosteroids, etanercept, and anakinra but was successfully treated with the anti-IL-6 monoclonal antibody, tocilizumab.