Nasal dermoid and sinus cysts (NDSC) are uncommon congenital anomalies that may have intracranial extension and can be associated with other anomalies. We identified 22 patients in a retrospective review of cases diagnosed with NDSC at our institution over the past 10 years. Nine (41 percent) had associated anomalies and ten (45 percent) had intracranial extension of the sinus. In half of the patients with intracranial extension, the sinus transversed either the cribriform plate or foramen cecum and attached to the dura; in the other half, the sinus extended to cysts within the falx or other brain structures. Of the patients with multiple anomalies, six (67 percent) had intracranial extension. Presurgical complications occurred in a total of eight patients (36 percent): two had meningitis, two had osteomyelitis, four had periorbital-nasal cellulitis, three had nasal abscess, and four had nose anomalies requiring rhinoplasty.