Abstract: Primary esophageal small cell carcinoma (PESCC) is a rare disease first described by McKeown in 1952. PESCC is characterized by high malignancy, distant metastasis, and poor prognosis. The incidence of PESCC has significantly increased worldwide in recent years. However, practice guidelines that concern the histological origin, clinical diagnosis methods, therapies, and prognosis of PESCC are still not well established because of the paucity of cases and lack of large prospective randomized research. This article aims to outline recent advances in the clinical and therapeutic aspects of PESCC as well as review the different opinions concerned to better understand PESCC and solve clinical problems.