Primary small cell carcinoma of esophagus (SCCE) is a rare disease with poor prognosis. The aims of this study are to review the clinical characteristics, treatment modalities, and outcomes of SCCE and to investigate the prognostic factors and optimal treatment options. Sixty-four patients diagnosed as SCCE in Sun Yat-sen University Cancer Center from 1990 to 2011 were retrospectively reviewed. There were 46 patients with limited disease (LD) and 18 with extensive disease. The median survival time (MST) and overall survival rate were calculated and compared by the Kaplan–Meier method and log-rank test, respectively. The prognostic factors were calculated by Cox hazards regression model. With a median follow up of 11.6 months, the MST of all the 64 patients was 12.6 months, 16.5 months for LD and 9.0 months for extensive disease. The 1-, 3-, and 5-year overall survivals were 52.5%, 20.9%, and 7.5%, respectively. In univariate analysis, patients with ECOG performance score <2 (P = 0.009), lesion length ≤5 cm (P = 0.009), T stage ≤2 (P = 0.004), LD (P = 0.000), and multimodality treatment (P = 0.016) had significant associations with MST. Multivariate analysis showed that ECOG performance score (P = 0.001), T stage (P = 0.023), limited-extensive stage (P = 0.007), and treatment modality (P = 0.008) were independent prognostic factors. Locoregional treatment combined with chemotherapy had a trend to increase MST from 15.3 to 20.0 months in LD patients (P = 0.126), while combined chemotherapy had a significant impact on MST in extensive disease patients (P = 0.000). SCCE is a highly malignant disease with poor prognosis. Patients might obtain survival benefit from the combination of locoregional treatment and systemic therapy. Prospective studies are needed to validate these factors.