Midaortic syndrome (MAS) is a rare condition characterized by stenosis of the abdominal aorta with or without the involvement of branch vessels. The majority of cases are thought to be idiopathic though MAS has been associated with a number of conditions including granulomatous vasculitis, neurofibromatosis-1 (NF-1), Alagille Syndrome, fibromuscular dysplasia (FMD), and Williams syndrome. Patients typically present with hypertension due to decreased renal perfusion. Less common presentations include renal insufficiency, heart failure, claudication, stroke, and abdominal pain. Imaging modalities help establish the diagnosis of MAS including duplex ultrasound, computed tomography angiography (CTA), magnetic resonance angiography (MRA), and angiography. Initial therapy focuses on medical management with antihypertensives prior to intervention. Invasive interventions are indicated when there is evidence of end organ damage or dysfunction such as decreased renal function, poorly growing kidneys, cerebrovascular accident, left ventricular hypertrophy or frank cardiac failure. Endovascular interventions may assist in diagnosis and may treat some lesions although reintervention rates are high. Most patients require some type of surgical intervention, and a variety of surgical options are available based on anatomic findings. Renal revascularization may be accomplished by renal artery bypass, autotransplantation, or renal artery reconstruction. Aortic lesions may be repaired using patch angioplasty or aortoaortic bypass. Mesenteric arteries do not typically require reconstruction as they are rarely symptomatic. More novel options include the use of tissue expanders to lengthen the aorta to allow for primary aortic reconstruction (TESLA) or the use of the meandering mesenteric artery as an autologous aortic bypass graft (MAGIC).