Introduction: Double aortic arch (DAA) is the most frequently encountered vascular ring malformation characterized by a complete encirclement of the trachea and esophagus by the aortic arch with a wide clinical spectrum. Objective: The aim of this study is to describe a case of DAA in adolesecents. Case Presentation: An 11-year-old boy was admitted for chronic productive cough which initially was diagnosed with pulmonary arterial hypertension. The final diagnosis was DAA which was established after chest computed tomography (CT) scan. Conclusion: Establishing a diagnosis of DAA in adolescents requires thorough understanding and clinical skills in performing diagnostic steps. The management of heart failure symptoms and other comorbidities is essential while preparing for its definite therapy.