A huge schwannoma, located in the retroperitoneal space, is found very rarely. The main purpose of this paper is to present the case of a giant retroperitoneal schwannoma which partly invaded L4 vertebral body. The secondary purpose is to summarize the case-report articles on retroperitoneal schwannomas. A circumscribed heterogenic tumour was revealed on transabdominal sonography. It extended into the right retroperitoneal space. CT and MRI revealed a paravertebral tumour in the size of 11 cm × 9 cm, which is causing a partial lysis of L4 vertebral body (15% of vertebral capacity), expanding intravertebral foramen and filling the right retroperitoneal space. A preoperative core needle biopsy was performed and a benign schwannoma was diagnosed. A complete surgical excision of the tumour was achieved by a two-step operation. During the first step, the neurosurgeons made L4 hemilaminectomy, facetectomy and a transverse process resection by posterior extended approach. The general surgeons excised the residual retroperitoneal part of the tumour by midline transabdominal approach 10 days later. The diagnosis of benign schwannoma was verified histochemically. There were no sign of tumour recurrence or spine destabilization at the six-month follow-up. In conclusion, although majority of giant retroperitoneal schwannomas can be completely removed performing one-step operation, a preoperative consideration about rationality of two-step operation should be mandatory when tumour destructs a part of vertebral body. Our case shows that the combined two stage complete surgical excision of a giant retroperitoneal schwannoma, eroding 15% of L4 vertebra’s osseous capacity, is effective and does not have any negative influence on spinal stability.