Scleredema is a rare connective disease which must be differentiated from scleroderma in childhood. Scleredema is characterized by thickening of the dermis of the neck, head, and upper trunk. We report a case of scleredema in an 8-year-old boy with coincident streptococcal colonization. The patient report demonstrates many of the common features of scleredema, including an associated streptococcal infection, a relatively benign presentation of illness, and the characteristic mucopolysaccharide intradermal staining on skin biopsy. The literature on scleredema is reviewed, focusing on the disease course, differential diagnosis, and an overview of the proposed three subgroups of scleredema. The association of scleredema to a prior streptococcal infection is explored, and a proposed autoimmune pathophysiology of the disease, as it relates to streptococcal infection, is presented. © 1994, Sage Publications. All rights reserved.