A 60-year-old male presented with a rare case of periventricular schwannoma. Imaging studies revealed a partially calcified, well-enhanced tumor in the periventricular area of the left frontal horn. The preoperative diagnosis was low grade glioma, but postoperative pathological findings revealed that the tumor was schwannoma. Most intraparenchymal schwannomas are benign, so total extirpation is usually curative. However, this uncommon neoplasm is difficult to distinguish from mimics, especially low grade gliomas, with only preoperative radiological findings or intraoperative pathological findings. Based on our experience and analysis of the characteristic radiological and pathological features in previous cases, we suggest that an accurate intraoperative diagnosis is possible. The key element is the combination of correct interpretation of the intraoperative pathology analysis and careful evaluation of the preoperative radiological studies. An appropriate intraoperative judgment is important, because the treatment, including the surgical management, would be totally different for schwannoma and glioma.