<p>Myasthenia gravis (MG) is characterized by antibodies against the neuromuscular junction causing fatigable weakness, while autoimmune premature ovarian insufficiency (APOI) manifests as amenorrhea and elevated levels of follicle-stimulating hormone in the absence of a genetic cause. We present a case of a 25-year-old female with a 3-year history of fatigable weakness and dysphonia accompanied by secondary amenorrhea. She is positive for anti-acetylcholine receptor antibody and underwent thymectomy revealing thymic hyperplasia. Post-thymectomy, hormonal replacement therapy (HRT) was initiated after the work-up revealed APOI. She eventually had improvement in her myasthenic symptoms and had a resumption of menstruation. We performed a literature review of articles on the occurrence of MG and APOI until October 2024. From 55 initial articles, 11 studies involving 14 patients met the inclusion criteria. MG manifestations developed at a mean age of 24.26 years while APOI presented at a mean age of 22.66 years. Thymectomy improved MG symptoms in 10 patients and resulted in the resumption of menstruation in 6 patients. Mutation in the autoimmune regulator (AIRE) gene was found to predispose 45–60% of women to develop APOI while polymorphism of the AIRE was postulated with an increased susceptibility to develop AntiAChR + MG. The co-occurrence of AntiAChR + MG and APOI suggests a possible autoimmune connection through thymic pathology. Thymectomy on AntiAChR + MG patients is still recommended; however, its benefit for APOI on patients with co-occurring AntiAChR MG needs further investigation.</p>