Purpose: To describe the optical coherence tomography (OCT) features of X-linked choroideremia (CHM). Design: Prospective, observational case series. Patients and Methods: Color fundus photography, fluorescein angiography, indocyanine green angiography, and OCT were performed for four patients (eight eyes) with CHM. Results: In areas of angiographic choriocapillaris loss, OCT revealed retinal thinning (mean, 124 μm [range, 97-150 μm) and increased reflectivity of the choroid. On the contrary, in areas of preserved choriocapillaris, retinal thickness was within normal limits (mean, 256 μm [range, 238-284 μm]; P < 0.001). Conclusion: OCT revealed a correlation between retinal thickness and choriocapillaris preservation in CHM.Intravitreal injection of triamcinolone acetonide might temporarily improve visual acuity in eyes with cystoid foveal edema associated with Coats disease.