Brain stem cavernous malformation(BSCM) is a kind of congenital vascular malformation. It mostly occurs in the region of pons, then midbrain but rarely medulla. BSCM can lead to many severe clinical consequences, sometimes even including death, due to its features of deep location, repeated hemorrhage and adjacency to important central neural structures. Microneurosurgery is the only effective way for the radical resection of BSCM. The characteristics of its epidemiology, pathology, clinical outcome, radiography and treatment were reviewed in this literature.