Idiopathic Tolosa-Hunt syndrome (ITHS) is a very rare cause of painful ophthalmoplegia characterized by unilateral orbital pain, ipsilateral oculomotor paralysis and prompt response to steroids. In this paper we report 4 additional cases of ITHS. This rare cause of painful ophthalmoplegia effects the cranial nerves due to a granulomatous lesion of unknown etiology in the cavernous sinus or superior orbital fissure. The International Headache Society redefined the diagnostic criteria for ITHS but it is still mostly a diagnosis of exclusion. Careful evaluation and follow-up is essential for diagnosis. Optimal therapy duration and dosage and prophylactic treatment in recurrent cases needs further research.