Background: Thymoma is an epithelial mass arising from the thymus. Most thymomas are located in the anterior mediastinum. Ectopic intrapericardial thymoma is very unusual; to date, only eight cases of pericardial thymoma have been reported. Among thymoma patients, 20% to 25% are associated with myasthenia gravis. However, postoperative myasthenia gravis occurs in less than 1% of cases. Here, we share a rare case of ectopic intrapericardial thymoma that developed postoperative myasthenia gravis six months after surgery. Case presentation: A 66-year-old woman visited the outpatient department due to productive cough and chest pain. Chest radiography showed increased soft tissue opacity over the mediastinum. A soft tissue mass in the pericardium and a ground glass nodule in right upper lung were noted using chest computed tomography. The diagnosis of thymoma, type B2, pT3N0M0, and stage IIIA and synchronous adenocarcinoma in situ of the right upper lung was confirmed after surgical removal. Six months later, the patient developed postoperative myasthenia gravis. Conclusions: Thymoma is rarely considered a differential diagnosis in pericardial tumors. Surgical removal with adjuvant radiation therapy should be performed considering the malignancy potential of thymomas and cardiac complications. In patients without myasthenia gravis, a small chance of postoperative myasthenia gravis remains. Patients should be carefully monitored for myasthenia gravis after surgery.