One patient of neurofibromatosis type I originating from the pterygopalatine fossa and salivary gland underwent surgery in our department. At the outset, the case was misdiagnosed as arising from salivary gland tumors, and surgery was immediately performed. The tumor was entered directly, which induced ferocious bleeding. The subsequent hemostasis with tamponade and compression caused intracranial venous sinus thrombosis, which induced irreversible intracranial complications and finally resulted in death. The experience regarding the diagnosis and treatment of the case was retrospectively reviewed and shared in this article.