The authors report an unusual case of a 50-year-old woman presenting with cavernous sinus syndrome, who had a cavernous sinus cavernous hemangioma (CSCH). The acute onset of her symptoms including pain of the right eye, blephaloptosis of the right eye, diplopia, and sensory disturbance of the right face was similar to those of Tolosa-Hunt syndrome. Magnetic resonance imaging and angiography showed a tumor in the right cavernous sinus. Although she showed improvement of the symptoms after receiving oral corticosteroids, follow-up neuroradiological investigations after a year from the onset revealed the mass in the right cavernous sinus which grew up in size. The histopathological findings obtained from the biopsy of the mass demonstrated a CSCH. Our findings suggest that the growth mechanism of CSCH could be progressive ectasia of vessels or their autonomous development at the edges of the lesions.