Nasal gliomas are extremely rare in neonates with an incidence of 1 in 20,000 to 40,000. They often are asymptomatic but can present with respiratory distress depending on the size and location of the tumor. A newborn female was prenatally diagnosed with a left nasal mass. After her birth, she was transferred to a local children's hospital for subspecialty evaluation and for diagnostic imaging. The mass was resected at 1 year of age. Pathology confirmed a nasal glioma. Several weeks after surgery, a nasal prosthetic device was applied to correct the nasal deformity caused by the pressure effect of the tumor. At almost 1 year of age, there was no evidence of metastasis or recurrence of the nasal glioma. The prognosis and outcome tend to be favorable. The rare case of a neonate with a congenital nasal glioma is presented.