BackgroundChoroidal osteoma is rare clinical entity of unknown etiology, characterized by formation of mature cancellous bone within the choroid. It typically affects young females, with no racial predilection. Vision loss occurs mainly due to photoreceptor degeneration secondary to decalcification and/or development of choroidal neovascularization especially if located at the subfoveal area.Case presentationOur case is 9-year-old Indian (Indo-Aryan) boy identified incidentally with clinical features suggestive of choroidal osteoma with marked diminution of vision. Spectral domain optical coherence tomography demonstrated high reflectivity from the choroid and atrophy of the overlying retinal layers and B-scan ultrasound demonstrated multiple highly reflective calcified lesions within the choroid.ConclusionAlthough available literature shows that the occurrence of this rare clinical entity is more commonly seen in young females, our case report has shown that it may be seen at a very early age. The treatment options are still not available if significant atrophy of retinal pigment epithelium has already occurred; however, vision loss due to associated choroidal neovascularization may be treated with currently available treatment options. In our case, the vision loss was due to the significant atrophy of the retinal layers. Choroidal neovascularization was not seen and our patient was advised to attend follow-up regularly.