Background: Basilar fenestration is a rare congenital anomaly. When present, it is commonly found at the proximal portion of the artery near the vertebrobasilar junction. Methods: This is a case report of a ruptured basilar fenestration aneurysm in a 47-year-male successfully treated with endovascular coiling. Results: A 47-year-old male presented with sudden onset headache, neck pain, blurry vision, nausea, vomiting, and diaphoresis. Cerebral angiogram revealed a saccular basilar fenestration aneurysm located at the vertebrobasilar junction measuring 3.1 x 2.6 x 3.4 mm with a 2.3 mm neck. Multiple coils were placed including Target 360 Nano 2mm x 4 cm (Stryker, Kalamazoo, MI, US), Target 360 Nano 1.5 mm x 2 cm (Stryker, Kalamazoo, MI, US), and Target Helical Nano 1.5 mm x 1 cm (Stryker, Kalamazoo, MI, US). A repeat angiogram revealed complete exclusion of the aneurysm with preservation of both vertebral arteries. Conclusions: A literature review was conducted on basilar fenestration aneurysms which included 158 patients from 39 studies. Overall, complete exclusion of the aneurysm was achieved in 75.8% of cases, with 22.4% of cases having residual flow and 1.8% of cases with unreported exclusion status.