Serum neuron-specific enolase (NSE) is a tumor marker for neuroendocrine tumors or tumors of neuroepithelial origin. While the clinical application of NSE in small cell lung cancer has been established, its role in small cell carcinoma of the uterine cervix is unknown. We examined serum NSE in six patients with cervical small cell carcinoma and 13 patients with cervical squamous cell carcinoma. Elevated serum NSE was noted in four of six patients (67%) with small cell carcinoma, but none of the 13 patients with squamous cell carcinoma. An extremely high NSE serum level (154.7 ng/mL) was found in one patient with recurrence of small cell carcinoma, suggesting its correlation with disease extension. Although further studies to evaluate its relevance to prognosis, treatment effect, and early detection of recurrence are in progress, this preliminary result seems promising.