We describe a 45 year-old man, with scleromyxedema associated to systemic manifestations, including myopathy. After 6 years of dermatologic manifestations, muscular weakness, with proximal predominance resulting in incapacity, was established. Corticosteroids plus cyclophosphamide treatment suppressed muscular symptomatology, and a second biopsy was normal. The common association between scleromyxedema and myopathy and other systemic alterations suggest that this entity should probably be considered as a systemic disease instead as a skin disease.