Scleromyxedema is a rare disease characterized by extensive mucin deposition with fibrosis, and is associated with a monoclonal gammopathy. Currently there is no consensus on optimal treatment of this potentially fatal disease because of the lack of randomized controlled trials and limited number of case reports. At the time of this writing, 24 cases were published reporting clinical improvement of scleromyxedema with intravenous immunoglobulin. Herein we report a case showing dramatic improvement of scleromyxedema symptoms, both cutaneous and extracutaneous (including the dermatoneuro syndrome), and review the use of intravenous immunoglobulin in the treatment of scleromyxedema. This is a single case. The rarity of scleromyxedema, especially the dermatoneuro syndrome, precludes impedes large trials. In conclusion, increasing evidence supports intravenous immunoglobulin as an effective and relatively safe treatment for both cutaneous and extracutaneous manifestations of scleromyxedema, including the dermatoneuro syndrome.