Dermoid cysts are embryonic lesions that are usually observed in the lateral eyebrow region and the midlines of the nose and neck; they contain an epithelial lining and adnexal structures, and are typically round or oval shaped. If ruptured, an inflammatory response can occur in the area surrounding the cyst, which can also induce pain, while the locations around intracranial and intraspinal extensions have a higher risk of inflammation; therefore, a definitive diagnosis is important. In this study, we report the case of a 6-year-old patient who had a remarkable, tadpole-shaped dermoid cyst that was unique at the time of presentation. It is easy to overlook or misdiagnose such cases, so every treating physician who is presented with a nonspecific appearing mass by a patient must acknowledge the possibility of a dermoid cyst, whereby evidence of a deep component may lead to intracranial complications. The authors removed the entirety of the appearing mass in the treatment of this specific case and a recurrence was not reported. Additionally, a sufficiently aggressive preoperative evaluation needs to be conducted to exclude the risk of complications.