We present a case of cytokeratin (CK) 20-positive large cell neuroendocrine carcinoma (LCNEC) presenting with multiple skin metastases as the primary manifestation. The patient was a 55-year-old man who presented with a one- month history of subcutaneous skin colored nodules of various sizes on his trunk. Pathologic examination of the skin revealed a nested and solid proliferation of large undifferentiated cells with vesicular nuclei and prominent nucleoli. Tumor cells were found to be immunohistochemically positive for CK 20, chromogranin A, synaptophysin, and CD56. Based on these features, the tumor was diagnosed as a large cell neuroendocrine carcinoma with multiple skin metastases. Computed tomographic (CT) imaging found metastatic foci in the liver, pleura, bone, and lymph nodes. We were unable to identify the primary site of origin. To the best of our knowledge, this is the first case of a large cell neuroendocrine carcinoma with a primary manifestation of multiple skin metastases
