We aimed to report the case of a Pakistani female who presented with MMD and hyperthyroidism, and the worldwide literature review of the case reports on MMD associated with hyperthyroidism. Methods: The study was carried out using PRISMA’s recommendations. An electronic search on PubMed, Cochrane Library, and Google Scholar was carried out. Articles explaining the association between Moyamoya disease and thyroid-related diseases were included. Results: A total of 31 studies were included in the review, with the preponderance of Japanese females. The review has systematically compiled in detail all the aspects of moyamoya syndrome and thyroid disorders- from detailed histories of the included patients to the treatment interventions used and their outcomes. Conclusion: The authors are with the suggestion that more cases of MMD and its association with other major factors should be presented in Pakistan as well as in all parts of the world.