Purpose : Intramedullary spinal lesions in the conus medullaris (CM), including tumors and vascular lesion, are rarely reported. We reported various MR features of intramedullary spinal cord lesions involving the CM including ependymoma, hemangioblastomas, dermoid cyst, ventriculus terminalis and spinal AVF and tried to discuss them for differential diagnosis. Materials and Methods: Six patients (male: female = 4:2, mean age = 44.3 year old) were enrolled from the clinical database of our institute from 2004 to 2010 and their radiological images and clinical symptoms were reviewed retrospectively. All patients had taken initial and postoperative MRI with contrast enhancement using gadopentate dimeglumine (Gd-DTPA). These images were analyzed by tumor size, location, signal intensity relative to the spinal cord, vascular flow voids, syrinx or cyst, edema and enhancement pattern. Results: Contrast enhancement was seen in all intramedullary masses. An eccentric enhancing nodule was noted in two hemangioblastomas and unusual peripheral rim enhancement with septation was seen in ventriculus terminalis. Patchy enhancement of the CM was observed in spinal arteriovenous fistula (AVF). Extensive cord edema adjacent to the intramedullary lesions was seen in four cases and syrinx was noted in three cases. Vascular signal voids were found in two hemangioblastomas and one spinal AVF. Conclusion: In evaluation of intramedullary spinal lesions in the CM, it is necessary to consider these unusual MR findings and discriminate various pathologies with prudence and caution.