Encephaloceles, while a common entity affecting 1:4000 live births, typically occur in the occipital region. Encephaloceles involving the frontal region comprise only 15% of all cases. Naso-orbital encephaloceles are rarely seen. Our case profiles a child born at term with an atrial septal defect (ASD), micrognathia, cleft lip, and frontonasal as well as bilateral naso-orbital encephaloceles. At birth the encephaloceles were undetected. During the cleft palate pre-operative preparation, the bilateral naso-orbital encephaloceles were diagnosed as dacrocystoceles for which the child underwent surgical repair. Misdiagnosis and loss to follow up lead to delayed surgical treatment until the child was almost two years of age; the right eye was near complete closure due to the increasing size of the encephalocele. This case highlights the importance of meticulous radiologic interpretation of midline nasal masses, as a correct diagnosis impacts clinical management and directs surgical repair.
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