Neurotropic keratopathy is one of the major refractory corneal disorders, which was first recognized in 1824. This category of diseases is caused by damage to the trigeminal nerve and the consequent loss of corneal sensation. It leads to various types of corneal disorder, including superficial keratopathy, persistent epithelial defects and corneal ulcers. In the present article, we review the pathobiology and prevention/treatment of neurotrophic corneal diseases. The goals of treatment of neurotrophic keratopathy are to prevent the progression of corneal damage to promote epithelial healing in case that the original damage in the trigeminal nerve or its braches is difficult. The therapy should be prompt and based on the clinical stage of the disease. Although at present, there are no accurate treatment methods for neurotrophic keratopathy, several effective treatments have been reported. Signals derived from TRP channels are involved in homeostasis of tissues by controlling cell behaviors, i. e., gene expression for inflammation, cell migration, or cell survival/cell death. Targeting TRP channel in the cornea is expected to contribute to the development of a new treatment method for neurotrophic keratopathy. Further study and clinical trial are need to establish this new treatment strategy.