Paragangliomas (PGs) are rare tumors of the head and neck that require a multidisciplinary approach to their management. The recent elucidation of the genetic and molecular etiology for this disease has led to significant advances in our understanding of this disease and has had an important clinical impact. Consideration of genetic testing is strongly suggested for selected patients and their family members. Surgical therapy remains the treatment of choice for most tumors, although a role for stereotactic radiotherapy is emerging. This review highlights some of the advances made in recent years regarding the diagnosis and treatment of PG.