A 23-year-old woman presented with a history of reduced vision and foreign body sensation in both her eyes. Ocular examination revealed the simultaneous presence of bilateral keratoconus and granular corneal dystrophy. To our knowledge, bilateral concurrent occurrence of keratoconus and typical granular corneal dystrophy has not been reported previously. In such a case, penetrating keratoplasty performed for keratoconus may also carry the risk of recurrence of the granular dystrophy.