We describe a case of nasopharyngeal carcinoma (NPC) with diffuse skull base bony destruction, resulting in obstructive hydrocephalus. Previously, these lesions were reported to be NPC with brain metastasis, without diffuse bony destruction of the skull base or hydrocephalus. We found no other cases of NPC with brain metastasis and diffuse bony destruction of the skull base resulting in hydrocephalus in the literature.