Painful ophthalmoplegia (PO) is an important presenting problem to ophthalmologists and neurologists. The etiological differential diagnosis is extensive, including different syndromes and causes (vascular, neoplastic, infectivous, inflammatory). Current neuroimaging techniques allow visualisation of the area of the suspected pathology. Some rare causes of PO, such as Tolosa Hunt syndrome with negative neuroimaging findings or ophthalmoplegic migraine remain till now of uncertain classification. Correct approach to the patient requires correlation to clinical data and careful monitoring, to avoid diagnostic mistakes, as the "history" of Tolosa-Hunt syndrome has underlined.