Background: A 19-month-old boy with recent Wilms tumor resection presented with ASIA B spinal cord injury secondary to rapid progression of a T12 epidural lesion suspicious for metastatic disease. Methods: The case is presented and the literature was reviewed for prior cases of Wilms tumor with spinal metastasis. Results: Emergent tumor debulking for spinal cord decompression via T11-L1 laminectomies with right T11-L1 facetectomies was performed. Allograft bone was placed to facilitate fusion. No direct connection to the renal tumor was appreciated on imaging or intra-operatively. There was no evidence of additional metastases. Pathology demonstrated similar histomorphology and immunohistochemical profile as the original left kidney tumor resection (Wilms tumor, favorable histology). Notably, no focal or diffuse anaplasia, or aggressive non-Wilms component such as clear cell sarcoma or rhabdoid tumor of the kidney, were identified. Treatment plan consisted of 25 Gy of radiation and 29 weeks of chemotherapy. At 6-weeks the patient had regained baseline lower extremity function with no bowel or bladder dysfunction. Conclusions: Spinal cord compression secondary to spinal metastasis of Wilms tumor in the absence of global metastatic disease is rare. Prompt identification, surgical decompression, and multimodality therapy is essential to prevent persistent neurological deficits.