Only 2 subtypes of primary cutaneous neuroendocrine carcinoma were recognized in the 2018 World Health Organization classification of skin tumors: Merkel cell carcinoma (MCC; also known as primary cutaneous neuroendocrine carcinoma) and endocrine mucin-producing sweat-gland carcinoma (EMPSGC).[1] Here, we report a case of high-grade neuroendocrine carcinoma with positive hormone receptors. Here, NR4A1 is linked to Merkel cell skin cancer.