Interrupted descending aorta (IDA) is an extremely rare congenital heart defect characterized by a complete loss of connection between the ascending and descending aorta. This condition is typically diagnosed in infancy or early childhood, but there have been very few cases reported in adulthood. Here, we present a unique case of an IDA in a 16-year-old patient with concomitant aortic stenosis (AS) and bicuspid aortic valve (BAV), making it an extremely rare scenario. This case highlights the importance of early diagnosis and appropriate management in patients with an IDA, particularly when in association with other cardiovascular abnormalities.