The coexistence of features of chronic cutaneous lupus erythematosus and scleroderma in the same skin lesions is very infrequent and has only been reported in 3 patients. This exceedingly rare condition has been named “sclerodermiform linear lupus erythematosus.” We describe a new case of this dermatosis. Although the cause remains obscure, possible explanations include mosaicism following Blaschko’s lines or the transfer of microchimerisms that mount a chronic graft-versus-host-like reaction. We suggest that these features may be a distinct clinicopathologic disorder characterized by an initial lichenoid reaction followed by the production of excessive, abnormal connective tissue.