BACKGROUND Epithelioid sarcoma is rare, represents less than 1% of all sarcomas, usually occurs in the extremities, and rarely presents as a primary sarcoma of the spine. Publications are usually single reports or case series. We aimed to undertake a systematic review of publications of cases of primary epithelioid sarcoma of the spine to evaluate clinical presentation, diagnosis, management, and patient outcomes. MATERIAL AND METHODS We searched studies on spinal epithelioid sarcoma in the PubMed database. Only studies with secondary epithelioid sarcoma or without effective data for analysis were excluded. Cases in which epithelioid sarcoma first invaded other sites and then affected the spine were also excluded. RESULTS Twenty-three patients from 13 studies were included in the study, aged between 14 and 65 years, and the sex ratio of female to male was 1: 2.29. The survival time was 18.7±13.8 months. The survival time of males was longer than that of females (22.9±14.4 vs 9.0±4.6, P=0.027). The onset age was linearly correlated with the size of the lesion (size=-0.161*age+11.841).The lesions located in lumbar vertebra had the worst prognosis. Postoperative radiotherapy had a statistically significant effect on survival time (P=0.040). CONCLUSIONS This systematic review identified 23 published cases of primary epithelioid sarcoma of the spine. Pain was the main presenting symptom, and tumor size increased with patient age. Female sex and primary location in the lumbar spine were associated with poor survival. Although surgery was the first-line treatment, postoperative radiotherapy and chemotherapy may improve clinical outcomes.