Eyelid tumors in about 80–95% of cases belong to benign lesions. Because of the complex anatomy of the eyelids and the resulting diversity of potential sites of initiation of the neoplastic process, as well as because of the morphological similarity of the observed lesions, making a correct diagnosis at the initial stage of the diagnostic process is challenging. Neural tumors originating from peripheral nerves and neuroendocrine cells are rare neoplastic lesions of the eyelids. The most common initially benign neural tumors include plexiform neurofibromas, solitary neurofibromas, and schwannomas. In contrast, malignant peripheral nerve sheath tumors and neuroendocrine tumors originating from Merkel cells predominate among malignant lesions. Histopathological examination of the excised lesion is often necessary to make a definitive diagnosis and, if necessary, to implement adjunctive treatment. Complete excision of the lesion reduces the risk of local recurrence and, in the case of lesions showing the risk of malignant transformation, reduces the probability of its occurrence. This article summarizes the current state of knowledge regarding neural tumors of the eyelids – rare but potentially dangerous lesions – systematizing knowledge regarding their pathogenesis, diagnosis, and treatment.