Nasal dermoid sinus cysts are uncommon congenital anomalies presenting either as cysts or sinuses. They are frequently associated with extension into the intracranial space, requiring craniotomy for adequate resection. At the Royal Children's Hospital in Melbourne, Australia, we have managed 25 patients with nasal dermoid sinus cysts over 8 years and present details of clinical features, preoperative assessment, and surgical management. Six patients presented with infection, including 1 with osteomyelitis. Four of our patients had intracranial extension of their lesions, and all were treated successfully with tailored investigation and appropriate surgical procedures. Insights into diagnosis, investigation, and surgery are offered to facilitate the management of these challenging lesions.