Elephantiasic pretibial myxedema is an extremely rare condition, affecting patients with thyroid disease. The clinical manifestation is difficult to diagnosis, and the treatment is challenging. Herein, the authors report on a case of a patient with Graves’ disease, who had a long-standing enlargement of bilateral legs, and histopathologic studies confirmed the diagnosis as; pretibial myxedema. The patient was treated with an antithyroid drug, iodine-131 (131I) irradiation, a potent topical corticosteroid, with occlusion, and compression therapy, however, the patient’s skin condition remained unchanged. This report aims to demonstrate a rare form of pretibial myxedema, to review the clinical presentations, differential diagnosis as well as therapeutic options.