Dysembryoplastic neuroepithelial tumors (DNETs) are benign supratentorial tumors based in the cerebral cortex. They usually are found in children and young adults with seizures that tend to become refractory to medical treatment. In the vast majority of cases resection results in good seizure control, and adjuvant therapy is not required. When tumors thought to be DNETs are not resected due to their proximity to eloquent cortex, lack of change in the clinical and neuroimaging features over time supports the diagnosis of DNET. The authors report on a patient in whom a pilocytic astrocytoma developed within a DNET, raising questions regarding the classification of these lesions and the need for lifelong clinical and imaging surveillance. This paper adds to the growing body of literature about the biological behavior of these lesions.