Presacral ganglioneuromas are so rare benign tumors that only 17 cases have been reported in the literature. They are abdominal masses growing slowly and differential diagnoses have to be considered. Surgical resection is important for definitive diagnosis because it represents the only therapeutic choice. Because of the benign nature of ganglioneuroma, adjuvant chemo- or radiotherapy is not indicated but regular follow-up is necessary for an early diagnosis of potential local recurrence. We report a case of a 64-year-old man with a presacral ganglioneuroma.