Primary carcinoma of the sphenoid sinus is a rare tumor that may present with dramatic neuro-ophthalmological-symptoms and signs of which spheno-cavernous syndrome is the best known clinical entity. The most frequently encountered histological types of the sphenoid carcinomas are squamous cell carcinoma and papillary carcinoma, in decreasing order of frequency. In this article, a papillary carcinoma of the sphenoid sinus associated with sphenoid sinus abscess is presented. We are not aware of previously reported papillary carcinoma of the sphenoid sinus associated with sphenoid sinus abscess presenting as a cavernous sinus syndrome.