Purpose: To report an unusual case of an eye with primary ciliary body lymphoma which came to enucleation allowing detailed histopathological examination. Methods: A 50-year-old man presented with a painful loss of vision in the left eye. The clinical, imaging, and immunohistopathological features of this case were reviewed. Results: The vision in the left eye was light perception. There were keratic precipitates, an irregular and thickened iris with neovascularization. Imaging studies disclosed a ciliary body mass extending into the anterior chamber. The eye was enucleated and immunohistopathological examination showed positive staining with CD20, BCL-2, MUM1, and CD10. Staining with BCL-6 was weak and S100 and HMB45 expressions were negative. Occasional CD3+ reactive T cells were present. The Ki-67 index was 80-90%. All these results suggested diffuse large B-cell lymphoma. Conclusions: Diffuse large B-cell lymphoma may primarily arise from the ciliary body and can develop without systemic or central nervous system disease.