Neurogenic tumors of the mediastinum are among the most common mediastinal tumors. They include nerve sheath, autonomic nervous system, and paraganglionic tumors and malignant small cell tumor. Neurogenic tumors arise primarily in the paravertebral sulcus. They are most frequently identified as asymptomatic lesions on routine chest X-rays. The majority of symptoms can be related either to compression of neural elements or other intrathoracic structures or to excess production of biogenic amines. Complete surgical excision performed by a thoracotomy approach offers the best opportunity for cure. In the case of tumors with extension into the spinal canal, a combined thoracic and neurosurgical approach should be performed.