Background <p>Corneal dystrophy is a group of hereditary disorders with different presentations and management between regions and ethnic groups. This study aimed to report the patterns of corneal dystrophy, the frequency of keratoplasty requirement, and the outcomes of performed keratoplasty among cases with corneal dystrophy at a tertiary eye hospital in Nepal.</p> Methods <p>Data of all patients presented at corneal clinic, Tilganga Institute of Ophthalmology, with the diagnosis of corneal dystrophy documented in the Electronic Medical Record (EMR) from 1st January 2017 to 31st December 2025 were identified, categorized, and analyzed regarding demographic status, corneal dystrophic subtypes, the needs for keratoplasty, and the outcomes of the keratoplasty performed.</p> Results <p>Among 264 eyes of 132 participants, 50.8% and 49.2% were male and female, respectively. The mean age ± standard deviation of the participants was 43.7 ± 20.5 years. Epithelial-subepithelial, stromal, and endothelial dystrophy accounted for 9.8%, 21.3%, and 63.6%, respectively. Fuchs endothelial corneal dystrophy (FECD) was the most common subtype, followed by congenital hereditary endothelial dystrophy (CHED) and epithelial basement membrane dystrophy (EBMD). In 264 eyes of 132 patients, 92 eyes (34.9%) were indicated for keratoplasty. The proportion of eyes requiring keratoplasty were 34.8%, 29.3%, 31.5%, and 4.3% in FECD, CHED, the stromal dystrophy group, and unspecified corneal dystrophy, respectively. Among the eyes that underwent keratoplasty, 71.4% had clear graft at the l2-month follow-up visit.</p> Conclusion <p>Fuchs Endothelial Corneal Dystrophy was the most common subtype of corneal dystrophies in the current study. However, the proportion of FECD cases requiring keratoplasty was significantly lower than that in CHED and the stromal dystrophy group. Outcomes of keratoplasty were satisfactory, as a large number of cases had clear grafts at the12-month visit.</p>