Developmental midline nasal masses including nasal dermoids (NDs), encephaloceles (EPHCs), and nasal glial heterotopias (NGHs) are a consequence of disrupted embryonal developmental processes in the frontonasal region. Surgery is the only method of treatment in order to prevent local and intracranial inflammatory complications as well as distant deformities of the facial skeleton. Due to their rarity, similar location, and clinical and radiological symptoms, meticulous preoperative differential diagnostics is mandatory. The aim of this thorough literature review was to present and discuss all clinical, histopathological, and radiological aspects of NDs, NGHs, and EPHCs that are crucial for their differential diagnosis.