Scleromyxedema is a generalized cutaneous mucinosis that may cause internal damage. This condition is frequently associated with monoclonal gammopathy. However, its physiopathological implications remains uncertain. The natural development of scleromyxedema is unpredictable and may lead to potentially fatal complications. Although there is no standardized treatment, intravenous immunoglobulins are considered the best method for treating scleromyxedema. The effects of this method of treatment on this condition are not well known, and it could be argued that intravenous immunoglobulins interact with the monoclonal gammopathy. This paper describes a case of scleromyxedema without associated monoclonal gammopathy that was treated effectively using monthly courses of treatment with intravenous immunoglobulins.