A 3-month-old baby presented with a whitish lesion over the right cornea since birth. Due to the clinical and ultrasonic characteristics of the lesion, a manual anterior lamellar keratoplasty was performed. Histopathological examination showed it to be a simple grade II epibulbar choristoma. Although dermoid tumours are usually considered as benign, some of them, depending on their location, can seriously affect the vision of a paediatric patient. Due to the high probability of amblyopia in these cases, a surgical procedure is mandatory. Anterior lamellar keratoplasty is recommended when the visual axis is compromised without intraocular implications.