This paper focuses on the diagnosis and management of developmental anomalies of the skin that may be seen early in life. Common locations include the head, nose, preauricular area of the face, neck, and spine. Those that occur in or near the midline can be more serious because of possible intracranial connections. Radiologic imaging of the areas of involvement is often important; computed tomography (CT) scans can delineate bony defects; whereas, magnetic resonance imaging (MRI) more clearly defines intracranial connections. Occult spinal dysraphism can be suspected when certain cutaneous signs are present.