Background: Juvenile myasthenia gravis is a rare autoimmune condition affecting children. Similar to myasthenia in adults, juvenile myasthenia gravis is associated with thymomas and many of these patients undergo thymectomy. However, there is no clear consensus on which patients could benefit from surgical intervention and how to manage refractory disease.Case Description: In this report, we describe a young boy who was diagnosed with myasthenia gravis and found to have stage IVa type B2 metastatic thymoma. Despite numerous pharmaceutical interventions, including neoadjuvant chemotherapy, his disease continued to progress. He ultimately underwent an extended radical parietal and visceral pleurectomy with decortication, total thymectomy, partial pericardial resection, and diaphragm resection and reconstruction. However, he had recurrence of his thymoma and was started on prednisone for palliative intent. Remarkably, within a few months he was noted to have interval decrease in his thoracic tumor burden and near resolution of his metastatic disease.Conclusions: Although spontaneous remission has been described in pediatric myasthenia, this report sheds light on possible management strategies post-thymectomy. This case additionally underscores the varied outcomes in patients treated for juvenile myasthenia gravis. Ultimately, this case, when added to both existing and future studies, will inform the development of guidelines on pediatric myasthenia gravis treatment.