Hughes-Stovin Syndrome is a systematic disorder generally revealed by deep venous thrombosis (DVT) and pulmonary artery (PA) aneurysms. Its pathogenesis is still unclear and there are no previously established diagnostic criteria or treatment guidelines. It is usually associated with Behçet disease. The aim of this study was to further enlighten this entity as it is often misdiagnosed. We report a case series of five patients with this rare pathology with a systematic literature review of all the previously reported cases.