Juvenile dermatomyositis (JDM) is a systemic autoimmune condition with myopathy. Gastrointestinal and pulmonary manifestations are rare presentation of JDM. Gastrointestinal perforation incidence in JDM is associated with vasculopathy and ischaemia. There are only few reported case of management of JDM with gastrointestinal complication. Management of such condition is challenging. We present a 21-year-old man with spontaneous descending colon perforation undergoing Hartmann’s procedure. He subsequently presented with recurrent retroperitoneal abscess at five and 30 months following the initial presentation which was treated with percutaneous drainage. A high index of suspicion is necessary in JDM patients presenting with acute abdomen