Objective To investigate the pathological and clinical significance of 46 cases of rectal neuroendocrine tumors(NET). Methods Retrospectively analyzed the clinical and pathological feature of 46 patients with rectal NET, and assessed possible interactions between different pathological grades and gender, age, tumor diameter, depth of invasion, lymph node and liver metastasis. Results Rectal NET appeared more frequently in males than in females. Most tumors located in middle and distal third of rectum. The positivity rates of immunohistochemical marker CgA, Syn, CD56 were 40.0%, 97.8%, 100.0%, respectively. The cases of Ki-67 positivity rate under 2%, ranged between 3%-20%, above 20% were 36, 6, 4, respectively. Different pathological grades were significantly correlated with age, tumor diameter, depth of invasion, lymph node and liver metastasis, but not with gender. Conclusion Rectal NET had nonspecific symptoms. Combined immunohistochemical staining, such as CgA, Syn and CD56, was important in the evaluation of rectal NET. Pathological grading might be very useful for prediction of invasion depth, lymph node and liver metastasis.