Intracerebral paragangliomas are tumors with a low incidence. Only 13 cases have been reported in the literature to date. This type of tumor shows characteristic histopathological features but unclear histogenesis. Owing to its low incidence, little knowledge exists concerning the best therapeutic strategy. We report on a 55-year-old male patient with the diagnosis of a paraganglioma of the cerebellum. We compare our patient's case to similar cases reported in the literature, describe the typical course of the disease, and discuss therapeutic options. No relapse of disease was observed in our patient within 2 years of diagnosis. Surgery and adjuvant conventionally fractionated external beam radiotherapy seem to be well tolerated and feasible. A review of patients reported in the literature and a comparison to our patient reveals a good prognosis for intracerebral paragangliomas. The optimal therapeutic strategy with regard to the benefit of adjuvant radiotherapy after total or subtotal resection has yet to be defined.