Pneumatosis cystoides intestinalis (PCI) in adult dermatomyositis (DM) is rarely described. This report aimed to describe the clinical features and prognosis of PCI in six adult patients with DM (four with anti-MDA5 antibodies, one with anti-SAE antibodies, and one with anti-TIF-1γ antibodies). Except for one patient with transient abdominal pain, the remaining five patients were asymptomatic. PCI occurred in the ascending colon in all patients, of whom five had free gas in the abdominal cavity. No patients received excessive treatment, and PCI disappeared in four patients during the follow-up. Additionally, we reviewed previous studies on this complication.