Glomus tumors (also known as paragangliomas or chemodectomas) are rare tumors originating from the autonomic nervous system. They are benign in the majority of cases and are characterized by a strong vascularisation and slow growth. Even though they are most common tumors in the middle ear, their incidence is low. They occur with a frequency of about 1 in 300000. They occure more common in women. The malignant potential is very low, but widely spread metastases can be found. Glomus tumors can develop spontaneously or as a part of an autosomal-dominant inherited syndrome. The most common symptoms are hearing loss, pulsatile tinnitus and pain. With the expansion of the tumor into surrounding structures other symptoms such as vertigo, paralysis of cranial nerves, headaches and ear bleeding may occur. The most common otoscopic finding is a red, vascular formation that arises from the hypotympanum, known as the rising sun sign. Complete surgical resection represents the only treatment option even though resection of locally advanced tumors regularly results in lesions of the lower cranial nerves (VI-XII) and major vessels that have a significant impact on patients quality of life. With adequate preoperative preparation in 80-90% of the cases the tumor will be entirely successfully removed. Additional treatments include radiotherapy and stereotactic radiosurgery that can be used as a palliative method or primary treatment method, especially in large inoperabile lesions.