Background: Ganglioneuroma is a rare benign tumor, which is usually located in the posterior mediastinum and retroperitoneum. Occasionally, it involves the paraspinal region with intraspinal extension and becomes dumbbell shaped. Dumbbell ganglioneuromas rarely affect the lumbar spine, and only nine cases of dumbbell lumbar ganglioneuromas have been reported previously. Case presentation: We are reporting an extremely rare case of a giant dumbbell tumor in the lumbar spine. We performed a one stage total resection of the tumor with posterior approach and long-segment fixation and fusion. This approach is different from previous methods. Histopathological examination of the surgical specimen confirmed the diagnosis of ganglioneuroma. Thirty months follow up showed a satisfactory outcome. Conclusions: Single-stage posterior tumor resection with fixation and fusion is effective and an appropriate choice for the treatments of lumbar dumbbell ganglioneuromas. © 2016 The Author(s).