BACKGROUND Small cell carcinoma of the larynx is a rare, poorly differentiated neuroendocrine carcinoma (NEC). It is often diagnosed late, with delayed initiation of adequate therapy. METHODS The data for eight patients were retrospectively analyzed with regard to clinical information, surveillance, and diagnostic and therapeutic strategies. RESULTS The histopathologic diagnosis was made after a mean of 2.88+/-2.52 months after the first symptoms occurred. Staging and exclusion of an extralaryngeal primary was completed 1.75+/-1.7 months after the histological diagnosis. Seventy-five percent (n=6) of the patients already had regional or systemic metastases. Systemic metastases were detected in 50% (n=4) of the patients by FDG-positron emission tomography/computed tomography. Primary (n=4) or postoperative (n=3) radiochemotherapy was performed 3.07+/-1.32 months after initial diagnosis. Overall survival (n=6) was 12.83 +/-3.76 months. CONCLUSIONS Studies providing high-level evidence cannot be expected because of the low incidence of small cell NEC. Efficient diagnosis should be followed immediately by combined radiochemotherapy. As with small cell bronchial carcinoma, small cell NEC of the larynx should be regarded as a systemic disease and be treated in a similar way.