OBJECTIVE To study the clinical characteristics, pathological features, diagnosis, therapy and prognosis of primary small cell carcinoma of the larynx (PSCCL). METHODS Six cases of PSCCL collected from 1990 to 2009 was retrospectively analyzed. The diagnosis was confirmed by pathological examination. Among six patients, one case belonged to stage III, and the others were in stage IVA. One case abandoned treatment; one case received chemotherapy; one case underwent supraglottic hemilaryngectomy and adjuvant chemoradiotherapy; one case underwent induction chemotherapy, radiotherapy and consolidation chemotherapy. Two cases received induction chemotherapy, concurrent chemoradiation and consolidation chemotherapy. The drug regimens included bleomycin, fluorouracil, cisplatin, etoposide and taxel for 3-6 cycles. The radiotherapy technique included conventional radiotherapy, CT-Sim and three dimensional conformal radiation therapy with (60)Co or 4 MV X-ray for 60 - 66 Gy during 6 - 7 weeks. RESULTS The time of follow-up was 3 - 24 months and the median was 13 months. Two patients applied with concurrent chemoradiation were alive without tumor. The patient abandoning therapy died of respiratory failure, and the others died of lung or liver metastasis after 8 - 12 months. CONCLUSIONS PSCCL is a disseminated disease, so the pretreatment evaluation is necessary. Concurrent chemoradiation is an ideal treatment model for this disease.