This report presents a case of a 22-year-old woman with bilateral giant choroidal caverns (CCs) in rod-cone dystrophy. Her best-corrected visual acuity was 20/80 in the right eye and 20/50 in the left eye. Fundus examination showed optic nerve pallor, narrowed blood vessels, and diffuse pigmentary changes. Fundus autofluorescence showed a central area of reduced autofluorescence surrounded by stippled autofluorescence. An electroretinogram showed decreased amplitude of both scotopic and photopic waves, consistent with a diagnosis of rod-cone dystrophy (retinitis pigmentosa). Optical coherence tomography revealed large CC and foveal atrophy in each eye. A giant CC alongside rod-cone dystrophy is a distinctive feature.