Background: The association between cerebral aneurysms and left atrial myxoma is known but rare. We described its pathogenesis, clinical presentation, diagnostic findings and treatment using a systemic review of the literature. Methods: MEDLINE via PubMed was searched for articles published until August 2022 using the keywords “atrial myxoma”, “cardiac myxoma” and “cerebral aneurysm”. Results: In this review, 55 patients with multiple myxomas aneurysms were analyzed, and 65% were women. The average age when aneurysms were diagnosed was 42.5 ± 15.81; most patients were less than 60 years old (86%). Aneurysms could be found before the diagnosis, at the same time as cardiac myxoma, or even 25 years after resection of the atrial mass. In our review, the mean time to diagnoses was 4.5 years. Our review estimates that the most common symptoms were vascular incidents (25%) and seizures (14.3%). In 15 cases, variable headaches were reported. Regarding management strategies, 57% cases were managed conservatively as the primary choice. Conclusions: Although cerebral aneurysms caused by atrial myxoma are rare, the long-term consequences can be serious and patients should be monitored.