Objective To study the characteristics of intramedullaiy tumors of embryonal origin, surgical treatment and outcome. Methods The data of 26 cases with intramedullary tumors of embryonal origin, including clinical features, pathological types and imaging characters of the tumor, surgical treatment and outcome, were retrospectively summarized. Among the 26 patients, apart from the neurological symptoms, lower-extremity deformities were seen in 7 cases, scoliosis in 1 case, lower midline cutaneous abnormalities in 3 cases. The tumors involving lumber section of the spinal cord (thoracolumbar + lumbar segments + lumbosacral) in 21 cases, account for 80.8% (21/26). The tumor in different kinds had its characteristic MRI appearance. All of the 26 patients underwent surgical treatment. Subtotal resection of the tumor was performed in 19 cases, most partial resection in 5 cases, partial removal in 2 cases. Results Of the 26 patients, 25 patients had improved neurologically when leaving hospital. Twenty-one patients were followed up from 6 to 82 (30.0 ± 8.2) months, 12 patients had a good recovery without any neurological defect, 7 patients could live independently, 1 patient needed care offered by others, 1 patient with malignant teratoma died at 6 months after operation. In the follow-up period, no patient needed the second surgery for recurrent tumor. Conclusions Apart from the neurological symptoms, other abnormalities such as lower-extremity deformities, scoliosis, lower midline cutaneous abnormalities could be appeared in patients with intramedullary tumors of embryonal origin. Most tumors involving lumber section of the spinal cord, could be diagnosed by MRI. Other spinal abnormalities frequently occur with the tumors. It is difficult to remove the tumors totally, while the surgical outcome is satisfactory.