Background:Moyamoya disease (MMD) is a steno-occlusive cerebral angiopathy. The incidence of intracranial aneurysms (IA) associated with MMD is high (3.4-14.8%) when compared to the general population (1-3%). IA in the middle cerebral artery (MCA) associated with MMD are rare, with only 25 cases described in the literature. Methods:Search on the PubMed platform, in English, with the MeSH terms “Moyamoya Disease”, “Intracranial Aneurysm” and “Middle Cerebral Artery”. There were 151 results, of which 7 were included in the review. Results:Sumi et al. and Larson et al. reported non-ruptured IA of the M1 segment, treated by surgical clipping and wrapping, respectively. Endo et al. chose to clip a ruptured IA in the M1 segment, followed by anastomosis between the superficial temporal artery and the MCA, excluding the IA . Liu et al. reported a non-ruptured MCA IA in a pediatric patient, treated through an encephalo-duro-arterio-synangiosis; the IA was excluded. Peltier et al. performed an indirect revascularization using the multiple bur-hole technique in a pediatric patient with non-ruptured IA in the M1 segment, excluding the IA. Rivera et al. opted for the conservative approach of a ruptured IA in the M1 segment, which was successful. Yan et al. described 19 IA in the MCA associated with the MMD, without specifying the adopted therapeutic. Conclusion:Due to the low number of cases, no recommendation can be made, and treatment should be individualized.