Keratoconus is known to be associated with a variety of ocular and systemic disorders. The common posterior segment disorders known to be associated with keratoconus are retinitis pigmentosa, macular coloboma, Leber’s congenital amaurosis, retinal aplasia and retrolental fibroplasias. Occurrences of keratoconus in association with tapetoretinal degeneration is rare and has been reported infrequently. Visualization of the fundus is often difficult in cases of keratoconus due to the associated refractive error and corneal opacities. This may make it difficult for the ophthalmologist to clinically diagnose associated macular degenerative changes preoperatively. We report a case of keratoconus who was diagnosed to have cone-rod dystrophy following successful corneal transplantation.