Rupture of an extracranial carotid artery aneurysm (ECAA) is a very rare and life-threatening condition. To obtain a comprehensive view of previous and current management of ruptured ECAAs (rECAAs), we analyzed all cases reported since 1940 and two of our own cases. We performed a comprehensive literature review of reports from the MEDLINE database on rECAAs and included two patients treated in our department. A total 58 reports of 74 rECAAs in 74 patients were analyzed. Their mean age was 50 years, and the male/female ratio was 2.2:1. Infection was the most common reported etiology (19 of 74; 26%), followed by connective tissue disorder (13 of 74; 18%), atherosclerosis (9 of 74; 12%), and previous trauma (5 of 74; 7%). For 28 patients (38%), information on the etiology was not available. Of the 74 patients, 24 (32%) had undergone reconstructive surgery, 10 (14%) had undergone endovascular treatment, 17 (23%) had undergone ligation, 2 (3%) had been treated conservatively, and 1 (1%) had died before receiving definite treatment. For 20 patients (27%), information on the treatment received was not available. The complications after reconstruction included carotid blowout (3 of 24 patients; 13%) and cranial nerve deficit (3 of 24 patients; 13%). Two patients (8%) had died of unrelated ECAA causes during long-term follow-up, and one patient (4%) had died of an ECAA-related cause within 30 days. After an endovascular approach, 1 of the 10 patients had developed a cranial nerve deficit. After ligation, five patients (29%) had experienced stroke, three of which were fatal. One conservatively treated patient had experienced no complications and one had died of an ECAA-related cause. The most common reported etiology for rECAA was infection. Reconstructive surgery was the most common approach and was safer than ligation, which carried a high risk of stroke. Endovascular treatment showed promising results, especially for distally located aneurysms; however, the number of patients has remained low. [ABSTRACT FROM AUTHOR]