We report a case of Tolosa-Hunt syndrome in a 4-year-old girl. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) revealed a left cavernous sinus involvement with internal carotid artery occlusion. Clinical signs included left ophthalmoplegia, convulsions and right hemiplegia. Etiopathogenesis of this syndrome is unclear but as in our case, response to corticosteroid therapy is spectacular and avoids unnecessary invasive diagnostic procedures. We underline the importance of cross-sectional imaging in the diagnostic approach. Several other diseases including tumors, vascular lesions and infections can have a similar clinical and/or imaging presentation. However, closely monitored evolution on imaging proves to be decisive in the establishment of final diagnosis of this syndrome.