Dermoid cysts are rare, benign neoplasms that develop when ectodermal tissue is ectopically included during neural tube closure. Only 0.7% to 1.8% of dermoid cysts occur intracranially, and these make up only 0.3% of all intracranial tumors. Definitive management of intracranial dermoid cysts is achieved with surgical excision, with the primary goal being removal of the lesion before rupture or infection occurs. The authors report the multidisciplinary management of a 6-month-old patient with a midline intranasal dermoid cyst with intracranial involvement successfully treated with surgical resection. The surgical approach included Neurosurgical access with a bifrontal approach to remove the intracranial portion, then Plastic Surgery accessed and removed the intranasal portion through a transcolumellar and infracartilaginous dissection. The entire cyst was successfully removed without rupture. The patient's postoperative course was without complication.Timely preoperative imaging and diagnosis were crucial to the success of this operation. When considering the possibility of a midline dermoid cyst with intracranial extension, it is essential to obtain proper imaging to distinguish between lesions on the differential diagnosis and to define extent of extension. Once diagnosed, surgical planning requires a multidisciplinary approach. Considerations for the optimal surgical approach to intracranial, intranasal dermoids include maximizing surgical exposure and ensuring an aesthetically pleasing reconstructive outcome.