The current evidence regarding immunotherapy plus targeted therapy in esophageal 
neuroendocrine carcinoma (NEC) is lacking. Camrelizumab is a programmed cell 
death protein 1 inhibitor. Apatinib is a selective tyrosine kinase inhibitor of 
vascular endothelial growth factor receptor-2. A 50-year-old female was 
initially diagnosed as primary esophageal NEC. Neoadjuvant chemotherapy and Ivor 
Lewis esophagectomy were performed (ypT3N0M0, stage Ⅱ). Twenty months after the 
surgery, an isolated mediastinal lymph node recurrence of NEC was recorded. The 
specimen revealed a positive expression of vascular endothelial growth factor 
and programmed cell death ligand 1. The diseased lymph node was slightly 
enlarged after two cycles of first-line paclitaxel liposome and S-1. Second-line 
apatinib and S-1 for 2 months also resulted in progressive disease. 
Subsequently, third-line camrelizumab plus apatinib was continued for 5 months. 
The patient demonstrated a progression-free status for more than 10 months 
following the combination therapy. Meanwhile, relevant studies of camrelizumab 
in gastric or esophageal cancer were briefly reviewed. Based on the current 
evidence, camrelizumab is a promising agent for esophageal cancer. More 
prospective trials are warranted before a definite recommendation could be 
drawn.