The major syndromes of chronic inflammatory myopathy (dermatomyositis and polymyositis) may have in certain instances extramuscular manifestations including pulmonary, cardiac, gastrointestinal, and of course, in the first instance, dermatological involvement. Here we review recent literature addressing these associated clinical areas of involvement. Malignancies, which may affect as many as 20% to 30% of adults with dermatomyositis, will not be addressed in this review.