Purpose: Small cell neuroendocrine carcinoma (SCNEC) of the oropharynx is an exceptionally rare and aggressive malignancy that is frequently misdiagnosed as squamous cell carcinoma (SCC). Due to its distinct biology and management, early recognition is critical. We present a rare case of metastatic oropharyngeal SCNEC and review the current literature to highlight key diagnostic and therapeutic considerations for surgeons. Methodology: We report the case of an 80-year-old male presenting with odynophagia, dysphagia, and a right level III neck mass. Imaging with MRI, CT, and FDG-PET demonstrated a posterior oropharyngeal mass with cervical nodal disease and distant liver metastases. Biopsy and histopathology confirmed high-grade small cell neuroendocrine carcinoma. A focused literature review was undertaken using PubMed, Scopus and Medline to identify published cases of oropharyngeal SCNEC, with emphasis on presentation, treatment, and outcomes. Results: Radiologically, the tumour mimicked advanced oropharyngeal SCC; however, biopsy revealed high-grade SCNEC. Given the presence of distant metastases at diagnosis, the patient was managed with palliative systemic chemotherapy (carboplatin and etoposide), achieving a partial metabolic response on post-treatment PET imaging. Literature review suggests that oropharyngeal SCNEC demonstrates early nodal and distant spread, poor prognosis, and limited role for surgery in advanced disease. Conclusion: Oropharyngeal SCNEC is a rare but highly aggressive malignancy that requires a fundamentally different management approach from SCC. Surgeons should maintain a high index of suspicion in atypical cases, as early histological diagnosis directly impacts treatment strategy and prognosis.