Overlap syndromes are a spectrum of diseases with clinical features of two or more classic connective tissue diseases. Polymyositis or dermatomyositis that is associated with features of systemic sclerosis, also called sclerodermatomyositis, is an uncommon overlap syndrome, with a rare antibody directed against the nucleolar antigen PM-Scl. This disease generally has good prognosis. We report a case of sclerodermatomyositis with an aggressive pulmonary and articular course.