A case of a 26-year-old woman with decreasing vision in her right eye, diplopia, headache, and galactorrhea is presented. Both CT and MR studies showed a large sphenoid fibroosseous lesion that had a ground glass appearance on CT and low-to-intermediate proton density and low T2-weighted and low T1-weighted signal intensities on MR. After contrast medium administration, this process diffusely enhanced on CT and MR. There was also an expansile mass in the sphenoid sinus that had intermediate proton density, high T2-weighted and low T1-weighted signal intensities compared with brain. This mass did not enhance but had an intensely enhancing, uniformly thin rim. The pathologic diagnosis was ossifying fibroma with a sphenoid sinus mucocele. There are only isolated reports in the literature of benign fibrosseous lesions causing mucoceles. This association is reviewed as are the findings in this case.