Intracavernous sinus arachnoid cysts are rare intracranial congenital lesions. When present, their anatomic location frequently results in cranial nerve palsy. A 15-year-old boy was admitted to our hospital with diplopia, which had gradually worsened over the previous several months. An arachnoid cyst was identified within the right cavernous sinus and fenestration surgery was performed. The patient recovered well and three months after the surgery, diplopia was disappeared. Surgical decompression of the intracavernous sinus arachnoid cyst is beneficial for symptomatic patients with this condition.