We present a case of juvenile type dermatomyositis and severe retinopathy. A 10-year-old girl presented with progressive weakness of proximal muscles, generalized rash, including heliotrope-type eyelid erythema, and bilateral visual disturbance. Laboratory data showed a markedly elevated serum creatine kinase and electromyography revealed a myogenic pathology. Funduscopic examination showed numerous cotton wool spots and macular edema. She developed massive rhabdomyolysis, generalized skin lesions, systemic edema, renal failure, and respiratory failure. After she received steroid pulse therapy, plasma exchange, and high-dose immunoglobulin, her general condition and visual symptoms improved. She remained well when we followed up her condition 5 years after the discharge.