IC is rare (the present cases account for 0.23% of all retrieved cases). Its chief clinical manifestations include headache, nasal obstruction, polydipsia and polyuria, and visual disorder. Lesions include solid, cystic, and mixed cystic and solid types. It is very difficult to resect the whole diseased region because this disease invades the cranial base, nasal sinuses and nasopharynx. Individualized treatments should be used according to lesion characteristics and invasion range, for example, stereotaxic interstitial brachytherapy. Radical resection or partial resection plus external beam radiation therapy produces better prognosis in IC than intracranial craniopharyngioma.