Background: Spontaneous isolated dissection of the iliac artery without involvement of the aorta is extremely rare, and has unique etiological backgrounds. The purpose of this study was to review 34 patients reported in the literature and to discuss etiological and therapeutic considerations. Methods and Results: Patients (21 male, 13 female) were identified in a PUBMED search since 1966. Median age was 45 years old (rage, 25 to 65 years). The patients were divided into five categories; 1) dissection associated with fibromuscular dysplasia (n=10), 2) associated with Marfans syndrome or other systemic disorders (n=6), 3) associated with atherosclerosis (n=10), 4) associated with pregnancy (n=2), and 5) associated with exercise (n=6). Four patients went into shock at presentation. Site of dissection was in the common iliac artery in 16 patients, and in the external iliac artery in 18 patients. Bypass surgery was performed in 20 patients, and an endovascular stent was placed in 5 patients. Seven patients had conservative therapy. There were two (6%) hospital deaths. Two patients developed another dissection shortly after treatment. There was no late death related to dissection. One patient had recurrent dissection in the contralateral iliac artery during the median follow-up of 12 months. Conclusions: Spontaneous isolated dissection of the iliac artery occurred resulting from various etiologies categorizedinto five subgroups. This vascular event is strongly associated with systemic disorders or certain physical conditions asso-ciated with hemodynamic, mechanical and/or hormonal stresses. Despite favorable outcomes, fatal dissection could occur,and awareness of this vascular event, careful follow-up and expeditious diagnosis and management are of great impor-tance to prevent catastrophic consequences.