We describe the clinical course and imaging features from two children diagnosed with focal cortical dysplasia and dysembryoplastic neuroepithelial tumor. These two children share similar pathology, however, this case report illustrates how their clinical presentation, management and imaging features differed with one treated primarily as a neoplasm, while the other as focal cortical dysplasia. These two patients illustrate the characteristics and challenges encountered with pediatric presentation of DNET and FCD. For patient 1, the clinical course, imaging and spectroscopic analyses reflected at early stage the ultimate pathological features of the lesion. In contrast, for patient 2, the early imaging features minimize what ultimately is predominately DNET.