AbstractPurpose: To carry out an epidemiological assessment of corneal dystrophies leading to corneal transplantation and to determine different subtype frequencies. Patients and Methods: In this retrospective study, pathological records of patients who had corneal transplantations other than endothelial keratoplasty between the years 2002 and 2014 were examined. To determine different subtype frequencies when corneal dystrophies led to corneal transplantation the IC3D classification of corneal dystrophies-edition 2 was used. Results: Of the 5867 eyes undergoing corneal transplant surgery during the study timeframe, 239 (4.07%) belonged to patients with corneal dystrophy. The most common age group was between 20 and 29 years (n=57; 23.8%). Macular corneal dystrophy was the most frequent corneal dystrophy subtype (n=117; 49%). Patients with epithelial and sub-epithelial dystrophies were significantly younger, and patients with Fuchs endothelial corneal dystrophy and lattice corneal dystrophy were the oldest age group when undergoing corneal transplantation. Conclusions: Based on our findings Macular corneal dystrophy was the most common corneal dystrophy subtype in patients scheduled for corneal transplantations other than endothelial keratoplasty. Keywords: Corneal dystrophy; Pathology; Corneal transplantation, Iran