A 49 year old woman with a fluctuating painful ophthalmoplegia and a history of polyarthralgia is reported. On examination, additional findings included a maxillary subcutaneous infiltration, sphenoidal sinusitis and pulmonary nodes. Lung and sinus biopsy demonstrated findings compatible with lymphomatoid granulomatosis. The patient was treated with prednisone and cyclophosphamide, with good response of neurological and dermatological findings.