Abstract: Objective To study the relationship between immunohistochemical markers and prognosis of patients with primary retroperitoneal extra-adrenal malignant paraganglioma (MPGL). Methods A retrospective analysis of immunohistochemical markers and prognosis of 17 patients with pathologicaldiagnosis as MPGL from January 1985 to January 2015 was carried out, and the relationship between them was analyzed. Results Of the 17 patients, 8 casesdied, 5 cses were Ki-67 positive, 9 cases were CgA positive, 10 patients with more than 3 kinds of chief cells and sustentcular cellsdetected to be positive by immunohistochemistry, and 8 cases withdistant metastasis. Of the 8dead patients, 4 were Ki-67 positive, 5 cases were CgA positive, 6 patients with more than 3 kinds of chief cells and sustentcular cellsdetected to be positive by immunohistochemistry, and 6 cases withdistant metastasis. Conclusion There is a certain correlation between immunohistochemical markers and prognosis of MPGL.