Background Intracranial aneurysms in children (≤18 years old) are rare, and their epidemiology is poorly understood. We present a prospective 14-year experience at our institution and review the literature to clarify the characteristics and outcomes of pediatric patients with intracranial aneurysms. Methods Review of medical records in the Johns Hopkins aneurysm prospective database and review of the literature since 1939 were performed. Results Of 1377 intracranial aneurysm cases at our institution from 1991 to 2004, 19 (1.4%) pediatric patients were treated using microsurgical or endovascular techniques. Male/female ratio was 2.2:1. Eleven percent occurred at the internal carotid artery (ICA) bifurcation, and 42% were located in the posterior circulation. Seven (37%) were giant lesions; 58% presented with subarachnoid hemorrhage. Patients in good Hunt and Hess grade (I-III) comprised 42%, and poor-grade (IV-V) patients comprised 16%. At a mean follow-up duration of 3.0 ± 3 years, 95% of patients demonstrated favorable outcomes with Glasgow Outcome Scale score of 4 or 5. In the literature, a total of 706 pediatric intracranial aneurysm cases have been described since 1939. The male/female ratio is 1.8:1. The ICA bifurcation was the location in 26%, and only 17% were in the posterior circulation. Twenty percent were giant lesions, and 80% presented with subarachnoid hemorrhage. Good- and poor-grade patients comprise 49% and 36% of the total cases, respectively. Favorable outcome was reported in 60%. Conclusions During recent decades, good outcomes after the treatment of ruptured and unruptured pediatric aneurysms have increased, reaching 95% in the current series. Pediatric intracranial aneurysms occur more commonly in male patients and have a predilection for the terminal ICA bifurcation.