Primary small cell carcinoma of esophagus (PSCCE) is a rare and highly aggressive neuroendocrine tumor, accounting for 0.05% to 3.1% of all esophageal malignancies. Its biological characteristics are similar to those of small cell lung cancer, with highly aggressive behavior and early dissemination tendency. It often metastasizes rapidly through lymphatic and hematogenous pathways.The prognosis is extremely poor, with a 5-year overall survival rate of less than 15%. There are no large-scale randomized controlled trials, and no standard treatment strategies have been established. In recent years, the treatment of limited-stage PSCCE has become a focal point of research. In traditional treatment paradigms, endoscopic therapy is feasible for very early-stage cases, while radical surgery serves as the primary approach for relatively early-stage patients. For locally advanced cases, two predominant treatment modalities are commonly employed in clinical practice: a surgery-based comprehensive treatment regimen and a radical chemoradiotherapy-centered therapeutic protocol, with no definitive conclusion yet reached regarding the optimal treatment strategy. Concurrently, emerging therapeutic strategies such as immunotherapy and molecularly targeted therapy have demonstrated remarkable clinical efficacy, thereby providing novel therapeutic opportunities for limited-stage PSCCE. This article aims to review the recent advances in the treatment of limited-stage PSCCE, summarize the current diagnostic and therapeutic landscape, and outline future directions in this field.