Neurotrophic keratopathy (NK) is a rare corneal condition resulting from impaired corneal sensory innervation. It leads to spontaneous, recurrent epithelial defects that can progress to corneal ulceration and perforationPerforation. Causes of NK are manifold. Clinically, NK is divided into three stages. In mild NK, surface irregularities with typical superficial punctate keratitisKeratitis and decrease or lack of sensitivity in one or more corneal quadrants are seen. Moderate NK is defined by epithelial defects with hypoesthesia or anesthesia of the entire corneaCornea. Severe NK entails additional stromal involvement (“ulcer”), potentially causing corneal perforationPerforation. Therapeutically, pre-existing topical therapyTherapy should be adjusted to avoid any potential epithelial toxicity. Frequent lubricating eyeEye drops are essential. In case of non-healing or recurrent epithelial defects, a bandage contact lens can provide protection from mechanical abrasion and thereby support resolution of the defect. Recombinant low molecular weight non-peptidic human nerve growth factor (NGF) is another therapeutic option which in clinical trials (Recordati study) has shown to improve epithelial healing in some patients. It however is costly and not readily available in every health system, which is why small molecules that mimic the effect of NGF are being investigated in clinical trials. These conservative options must be weighed individually against surgical measures such as amniotic membrane transplantationAmniotic membrane transplantation or tarsorrhaphy. KeratoplastyKeratoplasty is used in patients with severe NK and stromal ulceration. Corneal neurotisationCorneal neurotisation is an invasive, interdisciplinary treatmentTreatment option that provides a causal treatmentTreatment for NK and can restore corneal sensitivity.