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A 14-year-old male patient with Graves' orbitopathy presented with a downward gaze restriction in the left eye. Magnetic resonance imaging (MRI) revealed an edematous left superior rectus muscle. Retrobulbar injection of triamcinolone acetonide (20 mg) was administered in the left orbit. However, edema was still evident in the left superior rectus muscle on MRI, 3 months after the injection, and new inflammation was detected in bilateral inferior rectus muscles. The patient then underwent three cycles of steroid pulse therapy (1 cycle: methylprednisolone 10 mg/kg/ day × 3 days). One week after the steroid pulse therapy, eye movement was improved and the inflammation in the left superior rectus muscle and the bilateral inferior rectus muscles subsided on MRI. However, the patient noticed diplopia during upward gaze 2 months later, and MRI showed recurrence of edematous changes in bilateral inferior rectus muscles. The patient was treated with the same protocol of steroid pulse therapy. One month after the second steroid pulse therapy, ocular motility was improved and the inflammation in both inferior rectus muscles had almost resolved. This case illustrates the detailed clinical course of edematous extraocular myopathy in a pediatric Graves' orbitopathy patient, followed-up by successive MRI.