Objective:To explore the clinical characteristics,treatment and prognosis of basaloid squamous cell carcinoma of the esophagus (BSCCE). Methods:Clinical data of 44 patients with BSCCE were analyzed retrospectively. There were 31 patients in the traditional surgery group and 13 patients in extended mediastinal lymphadenectomy group,respectively. The relevant factors of these two groups and the survival-prognosis were investigated. Results:The tumor location and lymph node resection were associated with the operation mode (P < 0.05). The median survival time (MST) was 23 months,and the 1-,3-,5-year survival rates were 78.94%,62.82%,56.54%,respectively. COX regression analysis indicated that the depth of invasion was independent prognostic factors (P < 0.05). However,the survival was not significantly improved in patients undergoing extended mediastinal lymphadenectomy and adjunctive therapy (P > 0.05). Conclusion:BSCCE is a rare malignant tumor with high malignancy. Radical resection followed with adjuvant therapy is recommended while the effectiveness of extended mediastinal lymphadenectomy and adjunctive therapy requires further validation for BSCCE.