Anterior encephalocele with subcutaneous right facial nodule

Encephaloceles consist of heterotopic brain tissue that remains connected to the central nervous system. As such, these lesions can occur anywhere along the midline of the head, neck, and back. The clinical findings associated with an encephalocele are often cutaneous, prompting consultation with a dermatologist. Although abnormalities of the skin overlying the spinal cord are readily recognized by our specialty as markers for dysraphism, head and neck lesions may present a diagnostic challenge. We describe a case of an anterior encephalocele to increase awareness of this disorder and to emphasize the clinical findings that will assist with diagnosis. Our case is of particular interest because of the parasagittal location of the facial nodules and minimal actual midline involvement.