Nakajo-Nishimura syndrome (NNS) is a very rare hereditary disorder that has its onset in infancy with pernio-like skin rashes, and is accompanied by remittent fever and nodular erythema-like skin eruptions. The treatment of NNS is still under groping. Recently we encountered a case that was treated by corticosteroid and a humanized anti-human IL-6 receptor monoclonal antibody. As a result, the fever and skin rash was not improved sufficiently, and clinical symptoms of fat atrophy and joint contracture were gradually progressing. We herein report the effects of these agents and discuss the possibilities of new treatment direction.