Introduction: The association between ABO blood groups and intracranial aneurysms is not well-understood. Many co-morbid factors are associated with intracranial aneurysms. The prevalence of different blood groups and associations with the risk factors in patients with intracranial aneurysms are reviewed. Methodology: A retrospective analysis was conducted on patients presenting with intracranial aneurysms and undergoing active neurosurgical intervention at the Louisiana State University Health Sciences Center between 1 st January 1993 and 31 st December 2012. A total of 1248 patients were identified for the analysis. Data pertaining to demographics and clinical characteristics of these patients were extracted from the electronic patient records by two authors independently. A univariate and multivariate analysis was performed to investigate the association of ABO blood groups with risk factors in patients with intracranial aneurysms. Results: The most common ABO blood group in our study population was O (543 cases, 43.5%), followed by group A (525 cases, 42%). Blood group A (46% vs. 35.5% patients; P = 0.01) and blood group B (18% vs. 7% patients; P = 0.000) were significantly associated with the development of intracranial aneurysms in Caucasians and African Americans, respectively. We found smoking (P = 0.01) and hypercholesterolemia (P = 0.006) to be an independent risk factor for the development of intracranial aneurysms in blood group O and blood group A, respectively . Conclusion: Racial disparity in the distribution of blood groups and risk factor association with blood groups in the development of intracranial aneurysm needs to be considered. The findings from our study may be useful in identifying patients at increased risk of developing intracranial aneurysms.