Four subependymomas of the lateral ventricle were reviewed with regard to clinical presentation, neuroimaging features, treatment, histopathological features, and long-term follow-up. There were two male and two female patients ranging in age from 27 to 60 years (mean 48.3 years). While two patients presented with symptoms and signs of raised intracranial pressure, two others were found incidentally during neuroimaging investigations to have intraventricular tumors. Neuroimaging characteristics of these tumors included no paraventricular extension, iso- or hypodensity with minimal enhancement on computerized tomography (CT), or iso- or hypointensity on T1-weighted and hyperintensity on T2-weighted magnetic resonance images (MRI). The usual finding on MRI was of no or scarce contrast enhancement, but one case showed heterogeneous enhancement. Three patients underwent total resection of the tumor and one underwent partial resection. No patients received postoperative radiation therapy. All patients have been doing well 4.8 to 15.4 years (mean 8.8 years), after surgery. Although there are no absolutely specific features to distinguish these tumors from other intraventricular tumors preoperatively, subependymoma should be kept in mind for differential diagnosis, as this tumor may safely be removed without sacrificing contiguous normal tissue and with good long-term results.