OBJECTIVE: Tolosa-Hunt syndrome (THS) is a rare disease characterized by painful unliteral ophthalmoplegia and headache. THS is caused by granulomatous inflammation of the cavernous sinus, and its diagnosis is typically made by elimination and exclusion. The characteristic pain can typically be managed with steroid therapy, but relapses are common. Additional therapy is needed for refractory or recurrent cases. METHODS: Herein, using the electronic medical record with institutional review board approval, we report a case of a pediatric patient diagnosed with corticosteroid-dependent THS. The child was effectively treated with the tumor necrosis factor inhibitor (TNFi), adalimumab. We have reviewed the THS literature, including 2 adult THS patients who have been successfully treated with intravenous TNFi, infliximab. RESULTS: This is the first report in the scientific literature to effectively treat pediatric THS, and also the first such case to use adalimumab to successfully treat THS. CONCLUSION: Adalimumab, a subcutaneous TNFi, appears to be an effective treatment for corticosteroid-dependent THS.