Scleromyxedema is a rare cutaneous mucinous disease characterized by a generalized papular sclerodermoid eruption and systemic manifestations that can lead to significant morbidity and mortality. Although its etiology remains unknown, most theories focus on a pathogenic role by paraproteins; it must be noted, however, that nonparaprotein factors have been suggested to cause fibroblast proliferation and increased mucin production. Several treatment modalities including melphalan, cyclophosphamide, interferon alfa, and plasmapheresis have been suggested; however, further research is needed to prove treatment efficacy.