Abstract Intramedullary spinal cord metastasis (ISCM) is a rare condition. ISCM from esophageal cancer is extremely uncommon. We report a rare case of lumbar ISCM from esophageal cancer in a woman in her 70 s, initially diagnosed with clinical stage IVA (cT4bN1M0) esophageal cancer. She underwent definitive radiotherapy (60 Gy in 30 fractions) with concurrent chemotherapy of 5-fluorouracil and cisplatin (FP), followed by two additional cycles of FP chemotherapy. A complete response was maintained for 1 year and 7 months post-radiotherapy by endoscopy. However, the patient began to experience mild bladder and rectal dysfunction and back pain. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) revealed high FDG uptake in the spinal cord cavity at the L1 level and contrast-enhanced spinal magnetic resonance imaging (MRI) showed an intramedullary tumor corresponding to the areas of FDG accumulation. Based on the appearance of new lesions and elevated tumor markers, the lesion was diagnosed as ISCM from esophageal cancer rather than a primary spinal cord tumor, and palliative radiotherapy (20 Gy in 5 fractions) was promptly administered. Two months after radiotherapy, the patient’s neurologic symptoms improved, and she continued treatment with immune chemotherapy. To our knowledge, this is the first reported case of immune checkpoint inhibitors after the diagnosis of ISCM from esophageal cancer. Although rare, ISCM should be considered in cancer patients presenting with new neurological symptoms, and timely multidisciplinary intervention is essential for optimal management.