A 44-year-old woman developed a severe right frontotemporal headache, retro-orbital pain, and, later, diplopia owing to right sixth nerve palsy. The brain MRI demonstrated strong enhancement of the right cavernous sinus. The sixth nerve palsy and headache improved with steroid therapy after 6 weeks. At that time, she suffered right peripheral facial nerve palsy. Enhancement of the distal canalicular and labyrinthic segment of the right facial nerve was found on contrast-enhanced MRI. To our knowledge, this is a very rare case of Tolosa-Hunt syndrome with facial nerve palsy, with simultaneous enhancement of the cavernous sinus and facial nerve on contrast-enhanced MRI.