The recurrence of macular corneal dystrophy within a corneal graft has been described in a number of case reports. In this study, we reviewed 41 transplants in 31 eyes of 16 patients undergoing keratoplasty for macular corneal dystrophy confirmed by histopathological examination. The follow-up time ranged between 25 and 408 months from initial diagnosis. Six eyes of four patients had repeat keratoplasty because of clinical recurrence and visual impairment. Pathological confirmation was available in five of the six eyes. Peripheral clinical recurrence was observed in two more eyes. The size of the graft used was inversely related to the recurrence.