Over the last two decades a favourable course for treated or nontreated brainstem cavernomas has become possible with enhanced diagnostic tools and clinical experience, as well as minimally invasive microsurgical improvements. Currently, brainstem cavernoma can be treated microsurgically with excellent results and an acceptable morbidity rate. The preferred surgical route has progressively shifted from a dorsal to a lateral approach, but this remains dependent on the location of the lesion in the brainstem. Surgical evaluation and management of all cases of this rare disease should be performed by experienced teams from the outset.