We reviewed the surgical anatomy, different approaches to the brainstem, surgical indications and techniques that can be applied for the treatment of brainstem cavernous angioma. We also demonstrated the usefulness of surgical simulation with three-dimensional fusion imaging by presenting two surgical cases of brainstem cavernous angioma. Intraoperative monitoring of somatosensory evoked potential (SEP) and motor evoked potential (MEP) was useful in predicting postoperative neurological deficits. A brainstem incision should be made at a point where the angioma and hematoma are closest to the brainstem surface.