A female, eight-year-old, mixed-breed blue-eyed dog was presented for ophthalmic evaluation because its left eye had “changed color” one year previously. The before left eye was enucleated and submitted for evaluation. Histopathological analysis revealed an invasive neoplastic mass effacing most of the ventral aspect of the iris stroma. A diagnosis of an anterior uveal spindle cell tumor was made. Immunohistochemical results were strongly suggestive of a schwannoma, but some smooth muscle differentiation was also observed. Two and a half years after therapeutic enucleation there was no evidence of neoplasm recurrence or metastasis.