Objective To investigate the clinical and pathological features,immune phenotype,diagnosis,differential diagnosis of small intestine gastrointestinal stromaI tumors (GIST). Methods Clinical manifestations,pathological fea-tures and immunohistochemical characteristics were analyzed in 13 cases of small intestine gastrointestinal stromal tu-mors with review of the relative literature. Results Of 13 cases,age ranged from 34 to 76 years(mean 52 years),includ-ing 8 males and 5 females. There were 8 cases in jejunum,3 cases in ileum,2 cases in duodenum. In 13 patients,1 case was low risk,4 cases were middle risk,and 8 cases were high risk The diameter of tumors was 1.6-18 cm. Of 13 cases,there were 6 cases of spindle cell type,3 cases of epithelioid cell type and 4 cases of mixed cell type.Immunohis-tochemically,tumor cells were positive for CD117,DOG-1,Vimentin,but negative for Desmin, S-100. CD34 and SMA were positive in 5 cases,nestin was positive in 10 cases. The positive rate of Ki-67 was from+8%to+30%. Conclusion Small intestinal stromal tumors are rare tumors of alimentary tract, the prognosis was significantly worse in gastric GIST. Surgery is the principal and effective procedure for the tumors.