Clinically amyopathic dermatomyositis (CADM), a subtype of dermatomyositis (DM), is characterized as having the classic cutaneous manifestations of DM but with little or no evidence of myositis. The presence of anti-melanoma differentiation associated gene 5 (anti-MDA-5) autoantibodies, in the setting of CADM, is associated with rapidly progressive interstitial lung disease (RPILD). The use of extracorporeal membrane oxygenation (ECMO) in patients with RPILD has been well described in adults, but not in children. Herein we present a 14-year old patient with interstitial lung disease, initially of unknown etiology, whom we supported with ECMO in the acute phase of her illness for nearly two months during which the diagnosis of anti-MDA-5 dermatomyositis was established.