© 2015 by Nova Science Publishers, Inc. All rights reserved. Congenital nasal and paranasal masses are rare occurring in 1 in 20000 to 1 in 40000 live births. Commonly encountered masses are nasal dermoids, encephalocele and gliomas, the most common being nasal dermoids. They are due to developmental errors in embryogenesis at the level of the anterior neuropore, the central midface or the nasobuccal membrane and are associated with other congenital abnormalities. The differential diagnosis includes epidermoid cysts, hemangiomas, teratomas, neurofibromas, lipomas, lymphangiomas and nasolacrimal duct cysts. Presentation can range from benign cosmetic deformity to acute upper airway obstruction. Appropriate imaging with MRI and/or CT scan is essential for evaluation of intracranial extension and preoperative planning. Fine needle aspiration and biopsy are contraindicated. Treatment is mainly surgical either through an open external or an endoscopic endonasal approach and is multidisciplinary involving an otolaryngologist and a neurosurgeon.