BACKGROUND A 59-year-old woman with a history of nodular goiter developed thyrotoxic symptoms while on levothyroxine therapy. Her thyrotoxicosis persisted after levothyroxine withdrawal, so she was given methimazole and, once euthyroid, underwent near-total thyroidectomy. Histological examination revealed a nodular variant of Graves' disease. Proptosis, eyelid swelling and diplopia appeared 2 months after surgery. These symptoms worsened, and the patient was initially given four intravenous pulses of glucocorticoids, which resulted in a transient amelioration of her eye symptoms. After glucocorticoid withdrawal, however, the patient's eye motility worsened and there was a reduction of visual acuity in the left eye. She was then referred to our hospital for further advice and treatment. METHODS Complete thyroid and ophthalmological evaluation, computerized visual field analysis, CT scan of the orbits, routine blood tests, search for occult fecal blood, blood tests for hepatitis B and C virus markers, measurements of serum non-organ-specific autoantibodies and serum anti-TSH-receptor antibodies, and liver ultrasonography. METHODS Nodular Graves' disease with severe, active Graves' orbitopathy complicated by optic neuropathy. RESULTS Intravenous glucocorticoid therapy for 3 consecutive days, followed by once-weekly pulses of intravenous glucocorticoids over a 10-week period, and then by oral prednisone treatment on alternate days for 2 months. During the first 2 weeks of intravenous glucocorticoid therapy the patient received orbital irradiation. Therapy resulted in optimized visual acuity and a moderate improvement of soft-tissue inflammatory signs and symptoms, whereas proptosis and eye motility improved only slightly. The patient is now scheduled for orbital decompression and rehabilitative surgery.