Choroidal osteoma is a rare benign osseous tumor of the choroid usually affecting young healthy women. The diagnosis can be confirmed by the clinical appearance and with use of fluorescein or indocyanine angiography, optical coherence tomography, computed tomography or magnetic resonance imaging. A direct therapy of the tumor is indicated only for a few cases. The main reasons for vision loss are choroidal neovascularization, subretinal fluid and changes in the photoreceptor layer or the retinal pigment epithelium. Choroidal neovascularization and subretinal fluid in particular can be treated with laser therapy, photodynamic therapy or vascular endothelial growth factor inhibitors.