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The diagnostic criteria for Tolosa-Hunt syndrome (THS) were updated in 2013 in the 3 Edition of the International Classification of Headache Disorders. It is now possible to diagnose THS based on the presence of granulomatous inflammation demonstrated on magnetic resonance imaging (MRI) without confirmation by biopsy. No previous study has reported the use of arterial spin labeling (ASL) perfusion MRI for diagnosing THS. Here, we report a case of THS in which ASL was used in the initial identification and to monitor therapeutic response following steroid therapy.