INTRODUCTION: Juvenile dermatomyositis (JDM) is a rare immune-mediated disease, characterised by proximal muscle weakness and typical skin rashes1. We present a patient with severe JDM, to highlight the importance of a timely diagnosis and early initiation of treatment. CASE DESCRIPTION: A 9-year-old girl presented to the hospital due to asthenia, rash, generalized oedema, and inability to walk. At observation, the patient had dysphonia, dysphagia, proximal muscle weakness, petechial rash, skin ulcers, and anasarca. The levels of creatine kinase, aldolase, transaminases, and ferritin were elevated, and the NXP-2 antibody was detected. Prednisolone and methotrexate were started, followed by intravenous immunoglobulin. During the hospitalisation, the patient had an alveolar haemorrhage. Retinal vasculitis was also detected. Mycophenolate mofetil was added to the treatment. The patient had full resolution of myositis with progressive recovery of muscle strength, healed ulcers, and completely improved vision. DISCUSSION: This is an atypical presentation of JDM, without the typical skin lesions, but with several manifestations of severe vasculopathy, including retinopathy and alveolar haemorrhage. Early diagnosis and a multidisciplinary approach are crucial to improve prognosis.