An approximately 10-year-old, female Congo African grey parrot (Psittacus erithacus erithacus) developed progressive, unilateral exophthalmos and buphthalmos. Survey radiographs revealed a large, coelomic, soft tissue mass, which was confirmed on computed tomography scan. Aspirates of both the contents of the buphthalmic globe and coelomic mass were consistent with Cryptococcus species. Initial results were later confirmed with serum antigen latex agglutination and polymerase chain reaction testing, and the organism was then identified as Cryptococcus neoformans with DNA sequencing. During the course of 1 year, the bird was treated with combinations of oral terbinafine, fluconazole, and flucytosine, as well as intraocular amphotericin B. The coelomic mass dramatically decreased in size during the course of treatment, but the globe continued to enlarge. The bird died after exhibiting ataxia and seizures approximately 13 months after initial diagnosis, and necropsy confirmed colonization of the cerebrum and meninges with Cryptococcus. Cryptococcus remains a rare fungal disease of birds that is often refractory to treatment.