Isolated iliac artery aneurysm (IIAA), firstly reported in the 1820s, refers to a dilated artery disease limited in the iliac artery, and includes unilateral and/or bilateral common, external and internal iliac artery aneurysms, among which common iliac aneurysms account for 70% to 90%, internal ones for 10% to 30%, while external iliac artery rarely involved. IIAA is very rare clinically, with a prevalence of only 0.1% to 0.9%, and its etiology remains unclear. In clinical practice, it has occult manifestations and poor prognosis, and aneurysm rupture bleeding is the leading cause of death. This article reviewed the progress of IIAA in combination of its clinical characteristics, etiology, diagnosis and treatment.