Neurogenic neoplasms represent approximately 20% of the space-occupying lesions in the mediastinum. They are primary tumors originating from structures belonging to the peripheral or sympathetic nervous system, they are most commonly diagnosed in subjects in the age range between 10 and 30 years. Benign forms are significantly more frequent than malignant forms, an observation that was confirmed by our own experience; whether these tumors have a predilection for the male or female sex cannot be concluded from the various case series studied to date. Surgical resection can be considered the treatment of choice on the basis of the long-term results, which are greatly satisfactory in benign tumors and encouraging in the malignant variants.