Congenital corneal anaesthesia (CCA) is an uncommon condition difficult to diagnose. We report the case of a 20-month-old boy who presented with unilateral congenital corneal anaesthesia. The child was referred with a persistent corneal epithelial defect, unresponsive to symptomatic local treatment for over 10 months. Intensive topical treatment and strict corneal protection led to quick corneal healing. Congenital corneal anaesthesia occurs either alone or in association with neurological diseases or systemic congenital abnormalities. It is important to search for corneal anaesthesia in children with chronic ulcerations of the cornea and self-inflicted injuries. Early diagnosis and treatment are important due to the risk of poor visual prognosis. Management of CCA should aim for the prevention of epithelial defects and is a life-long process.