Lichen myxedematosus is a chronic, inflammatory, systemic dermatose characterized by dermal musin deposition and increased fibroblasts in the absence of thyroid dysfunction. It is usually seen together with paraproteinemia. It is clinically classified as scleromyxedema (papular mucinosis), localized lichen myxedematosus, and atypical lichen myxedematosus. Etiopathogenesis of the disease which is very difficult to treat is still unknown. Herein, we present a case of atypical scleromyxedema without monoclonal gammopathy mimicking angioedema, rapidly developing and responding to steroid treatment.