Surgical resection of intramedullary tumors remains technically challenging. The role of intraoperative ultrasound and contrast-enhanced ultrasound (CEUS) in these surgeries has not yet been well defined. This study was aimed to evaluate the potential of intraoperative ultrasonography, especially CEUS, in visualizing intramedullary spinal cord tumors, and to assess the values for improving surgical outcomes. This prospective study recruited 14 patients with intramedullary spinal cord tumor. All patients underwent tumor resection with intraoperative ultrasound and CEUS. The first ultrasonographic scanning was performed before the dural incision. After the dural incision, further ultrasonographic scanning was performed over the surface of the spinal cord. During and after the tumor removal, repeat ultrasonographic scanning was conducted to assess the extent of surgical resection. Follow-up data were collected and analyzed. Seven patients had small tumors involving 1 or 2 spinal segments, and 7 patients showed relatively large tumors involving ≥3 spinal segments. Intraoperatively, ultrasonography was able to visualize the lesion clearly in all cases. Using intraoperative ultrasound with CEUS, the tumor boundaries were clearly defined. After an average follow-up period of 15.93 months, neurologic function improved in 10 cases (71.4%) and remained unchanged in 4 cases (28.6%); there was no deterioration in individual neurologic function. Intraoperative ultrasound is of great value for localizing lesions and determining the extent of dural opening and myelotomy, therefore reducing the invasiveness of spinal surgery for intramedullary tumors. CEUS helps to delineate the tumor margins and perfusion features.