A 50-year-old woman presented with gradually increasing right-sided facial numbness. Neuroimaging revealed a lesion in the right cavernous sinus mimicking meningioma. The resection of the right cavernous sinus neoplasm was implemented via endoscopic endonasal approach under general anesthesia. Histological examination of the surgical specimen revealed adenoid cystic carcinoma. Adenoid cystic carcinoma in the cavernous sinus is extremely rare as a primary lesion and challenging to manage. Little data exist to guide treatment when this tumor extends to involve the structure of cavernous sinus. Our study illustrates that endoscopic endonasal approach is a good choice for resection of the tumor in the cavernous sinus.