Retinopathy has been described in dermatomyositis but visual loss is rare. We describe an unusual case of dermatomyositis where retinal complications resulted in permanent and profound central visual loss. Pulsed intravenous and oral methylprednisolone and cyclophosphamide therapy controlled the systemic disease but the macular and optic disc damage was irreversible. Early diagnosis and aggressive immunosuppressive therapy may help to prevent and control severe and potentially blinding intraocular inflammation. We emphasize the need for close co-operation between ophthalmologist and physician in the management of this condition.