Isolated iliac artery aneurysms are uncommon, comprising less than 2% of all abdominal aneurysmal disease. Although they have a fairly innocuous natural history, when they have attained a large size they carry a significant risk of rupture. Rupture is associated with significant morbidity and mortality. Therefore, an early diagnosis and treatment are crucial. Over the last decade, interventional treatment options have become established alternatives to open surgical repair. These guidelines aim to review the pathogenesis, natural history, and presentation of isolated iliac artery aneurysms including a description of imaging and interventional treatment strategies.