Painful ophthalmoplegia is an unusual presentation of malignant lymphoma. Moreover, spontaneous remission and exacerbation of it has quite rarely been reported. We present a patient who showed spontaneous remission and exacerbation of the painful ophthalmoplegia. Brain MR imagings showed infiltrating, iso-intensity mass a, TlWl and T2WI, and strong enhancement with gadolinium on TIWI in parasellar area. Malignant lymphoma of diffuse large cell type was found in cervical lymph node biopsy.