Juvenile dermatomyositis is an uncommon autoimmune disease with classic heliotrope discoloration of the eyelids, erythematous skin rash of joints, and proximal muscle weakness. It is most frequently sporadic and only rarely familial. We present juvenile dermatomyositis in a 5-year-old brother and a 3 1/2-year-old sister, both are very responsive to corticosteroids. Familial dermatomyositis can occur in different family members, and even dermatomyositis and polymyositis can coexist in the same family.