Ganglioneuromas are rare, benign, slow-growing tumors originating from sympathetic ganglion cells. The most common locations are the posterior mediastinum and retroperitoneum. Pelvic ganglioneuroma is very rare. The case of a 12-year-old girl with presacral ganglioneuroma is reported. The importance of considering a confident preoperative diagnosis by fine-needle aspiration biopsy is stressed.