Retroperitoneal emphysema represents a pathological situation with an abnormal amount of air in the retroperitoneal tissue. The diagnosis is made radiologically.The cause is mostly an iatrogenic complication of different diagnostic or therapeutic procedures. The most common cause is a perforation after endoscopic retrograde cholangiopancreatography (ERCP). In cases of clinically suspected complications after a procedure prompt diagnosis with computed tomography (CT) scan of the abdomen and an interdisciplinary decision on the suitable therapeutic measures is warranted, as immediate therapy reduces morbidity and mortality of patients. For selecting the ideal therapy it is essential to understand the underlying pathophysiological mechanism of retroperitoneal emphysema and to take the clinical situation of the patient into account. Thus periampullary or bile duct lesions facing the retroperitoneum can be treated conservatively in clinically stable patients, whereas in unstable patients with abscess formation, interventional radiological or endoscopic procedures are indicated. In cases of a duodenal lesion an endoscopic closure of the perforation can be performed in stable patients but if the patient shows signs of peritonism or if the clinical situation deteriorates, operative therapy is necessary.