Neurotrophic keratopathy is a degenerative corneal disease induced by an impairment of trigeminal nerve. Impairment of loss of corneal sensory innervation is responsible for corneal epithelial defects, ulcer, and perforation. In the present report, we reviewed the pathogenesis, diagnosis, and therapeutic aspects of this disease. An accurate history and clinical examination, including the function of cranial nerves, together with the clinical features of the ocular surface are essential for a prompt diagnosis. The evaluation of the corneal sensitivity and tear film function are important diagnostic steps as well. Specific medical and surgical treatments, based on the clinical staging of the disease, are often able to halt its progression. Future developments in the medical treatment including the administration of neuropeptide and growth factors are presented.