Appendiceal cancer is an extremely rare malignancy, and its metastatic spread to the brain is even more unusual. We describe a 47-year-old female who presented with a rare cerebral appendiceal carcinoma metastasis, a case that is further remarkable for representing the first histologic diagnosis of primary medullary carcinoma in the appendix. Based on a comprehensive review of the English literature using PubMed, Embase, and Google Scholar, only six other cases of cerebral appendiceal metastases have been described.