Malignancies with unknown primaries contribute to a small yet significant percentage of overall tumors. Neuroendocrine carcinomas, a rare disease with a poor prognosis, have been known to present as an unknown primary. Treatment consists of cytotoxic chemotherapy but given the latter's high toxicity profile new treatment options are being explored. In this case report, we describe a case of a patient with poorly differentiated neuroendocrine carcinoma of unknown primary treated with compassionate oral everolimus after his refusal of intravenous chemotherapy.