Ganglioneuromas arise from the neural crest and are highly differentiated and benign. The case of a 43-year-old female who presented with a 6.5 cm primary extra-adrenal retroperitoneal ganglioneuroma (RGN) is presented, and the relevant English literature from the last decade is reviewed. Histology showed mature ganglion cells and nerve fibers without any malignancy (S-100 and neuron- specific enolase [NSE] positive). Hospitalization lasted four days. The patient has shown no signs of recurrence. Radical excision of the tumor is unnecessary, especially when vascular structures are endangered. RGN-related hospitalization is short and the prognosis is good.