We report two cases of pleomorphic xanthoastrocytomas of the retina. The immunoreactivity to CD68, Fe (Pearls' technique), astrocytes (argentic technique), and antibodies to glial fibrillary acidic protein was tested in tumor cells. Both neoplasms contained cells immunoreactive for glial fibrillary acidic protein and CD68. These tumors, observed in the CNS, first reported by Kepes et al. were found in the retina as well-circumscribed masses with a cystic component. Many large pleomorphic cells contained vesicular and enlarged nuclei with a homogeneous eosinophilic cytoplasm, prominent nucleoli, and calcium deposits. Some cells had a markedly swollen lipidized cytoplasm, whereas others were spindle-shaped and contained multiple nuclei. These findings suggest that our two specimens have an astroglial lineage and are similar to pleomorphic xanthoastrocytomas of the CNS brain and spinal cord. Both reported cases were in female patients who were in their 20s and had glaucoma. After 10 years both are free of disease.