Tumors arising in the brain stem, comprising the midbrain, pons, and medulla oblongata, are now recognized as distinct clinico-pathological entities. Advances in neurosurgical techniques have made surgery not only feasible but the treatment of choice for some of these tumor types. Previously the mainstay of treatment, radiotherapy is now used more selectively. This article reviews the current state of knowledge with regard to tumors arising in the brain stem, the therapeutic options available for each, and provides recommendations with regard to management.