To evaluate indications for paediatric keratoplasty in recipients aged ≤16 years and assess long-term clinical outcome. Recipients were identified from records of the Danish Cornea Bank. Data were collected from patient journals, clinical follow-up examinations and questionnaires and stratified into pre-, peri- and postoperative variables. Diagnoses were classified into acquired traumatic, acquired nontraumatic and congenital groups. Recipients were divided into groups of <8 and ≥8 years. Data were analyzed using relative percentages and Kaplan-Meier survival plots. Thirty-three out of sixty identified recipients (73 keratoplasties in 63 eyes) were invited. Twenty-four accepted, seven still attended follow-up in our clinic. Follow-up data reached 95% of the eligible recipients (median follow-up 11 years). Twenty-three per cent were <8 years and 77%≤8 years. Diagnoses were mainly acquired nontraumatic (69%), acquired traumatic (12%) and congenital (7%). Indications were primarily optical (52%) or tectonic (41%). Graft survival was best in the acquired nontraumatic group (except regrafts) (median survival 15-20 years) and poorest in the regraft subgroup as well as the acquired traumatic and congenital groups (median survival 1-2 years). Graft failure was higher in the youngest with predisposing risk factors and in combined procedures. In terms of indications, visual improvement and eye preservation was achieved in 70%. Paediatric keratoplasty was successful regarding indication. Graft survival was best in the acquired nontraumatic group and poorest in the congenital group. Vascularization and/or combined risk factors, additional surgeries and young recipient age influenced negatively on graft survival.