<p>Thymoma-related aplastic anemia is a rare entity. This article retrospectively analyzes the clinical features and treatment course of a patient who developed aplastic anemia (AA) post-thymectomy, complemented by a systematic review of relevant literature. A 47-year-old female was diagnosed with thymoma, myasthenia gravis (MG), and severe AA (SAA). SAA onset occurred two weeks after total thymectomy, and the patient ultimately succumbed to concurrent COVID-19 infection following allogeneic hematopoietic stem cell transplantation (allo-HSCT). We also reviewed the clinical characteristics, treatment strategies, and prognosis of 47 thymoma-related aplastic anemia patients reported in the literature. AA may present prior to thymoma diagnosis, concurrently with thymoma, or post-thymectomy. Some patients progress to pure red cell aplasia (PRCA) and/or megakaryocytic aplasia, often following prior chemotherapy or radiotherapy. Similar to Good syndrome and PRCA, thymectomy fails to alleviate AA, and spontaneous improvement is rare. Treatment options for thymoma-related aplastic anemia include cyclosporine A (CsA) monotherapy, CsA combined with glucocorticoids, thrombopoietin receptor agonists (TPO-RAs), and allo-HSCT. However, regimens of cyclophosphamide plus methylprednisolone and glucocorticoid monotherapy show limited efficacy. The overall one-year mortality rate is alarmingly high at 29.8%. For young thymoma-related aplastic anemia patients with SAA and suitable donors, allo-HSCT remains the preferred treatment.</p>