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Mid aortic syndrome (MAS) is a rare condition often presenting with severe hypertension. It is characterized by narrowing of the abdominal aorta. We here describe a case of complete occlusion of the abdominal aorta presenting in infancy. This child presented at four months of age with heart failure and hypertension. CT angiogram showed total narrowing of the abdominal aorta. This was initially felt to be too severe for surgical treatment and he was planned for palliative care. We were however able to improve his blood pressure with antihypertensive agents and he underwent succesful angioplasty at five and a half months of age. He has required three further angioplasty procedures and still needs two antihypertensive agents to control his blood pressure. His renal function remains normal and at age six years he has excellent quality of life with normal growth and development. This case illustrates that the combination of medical treatment and angioplasty can give an excellent long-term treatment response even in children with extremely severe MAS. This boy now has normal blood pressure and has experienced normal growth, development and quality of life.