Dysembryoplastic neuroepithelial tumors (DNET) are usually benign lesions that arise in cortical regions and are discovered after new onset of seizure. These lesions have many different imaging characteristics. We report a patient with a presumed low-grade medial temporal lobe lesion that was followed for many years without any change in size or imaging characteristics. This previously non-enhancing tumor evolved to become contrast enhanced on routine imaging without apparent tumor growth. The patient underwent surgery, and the pathology was confirmed as a DNET with no atypical changes. This case demonstrates the potential that DNETs may exhibit a changing MRI pattern over time. Natural history, imaging characteristics, and management are reviewed.