Neuroendocrine neoplasms are a rare but challenging oncological entity. They originate from neuroendocrine cells, which are located in different organs such as the lungs, the gastrointestinal system, and the skin and serve as modulators of appetite, airway chemoreceptors, and receptors for tactile stimuli. According to the European Neuroendocrine Tumor Society, well-differentiated low-mitosis tumors (grades 1 and 2) are referred to as neuroendocrine tumors (NETs), whereas the poorly differentiated, high-mitosis neoplasms such as Merkel cell carcinoma (MCC) of the eyelid are classified as high-grade (grade 3) neuroendocrine carcinomas (NECs) (Table).1 We present for the first time, to our knowledge, a clinical-histopathological correlation of a patient with a primary, well-differentiated NET of the eyelid.