Dermatomyositis (DM) is rare systemic inflammatory disease with typical skin manifestations and muscular involvement. Various skin lesions can accompany this disease, such as Gottron's sign, Heliotrope rash, mechanic's hands, V sign and shawl sign. Scleredema is a very rare skin manifestation in DM. We report a case of DM in a 63-year-old woman, who had scleredema on her thighs. A diagnosis of DM was established by clinical manifestation, elevated muscle enzyme levels, electromyogram measures, and muscle biopsy findings. She was successfully treated with the immunosuppressants methotrexate, cyclosporine, and steroids (low dose).