Chiari malformation (kee-AH-ree mal-for-MAY-shun) is a condition in which brain tissue extends into your spinal canal. It occurs when part of your skull is abnormally small or misshapen, pressing on your brain and forcing it downward.
Chiari malformation type I develops as the skull and brain are growing. As a result, signs and symptoms may not occur until late childhood or adulthood. The most common pediatric form, called Chiari malformation type II, is present at birth (congenital).
Chiari malformations (CMs) are structural defects in the cerebellum, the part of the brain that controls balance. Normally the cerebellum and parts of the brain stem sit in an indented space at the lower rear of the skull, above the foramen magnum (a funnel-like opening to the spinal canal). When part of the cerebellum is located below the foramen magnum, it is called a Chiari malformation.
CMs may develop when the bony space is smaller than normal, causing the cerebellum and brain stem to be pushed downward into the foramen magnum and into the upper spinal canal. The resulting pressure on the cerebellum and brain stem may affect functions controlled by these areas and block the flow of cerebrospinal fluid (CSF)— the clear liquid that surrounds and cushions the brain and spinal cord—to and from the brain.
So basically it looks like this: 
So, a mere millimeters can cause a lifetime of problems! Now if you notice in the picture above showing the Chiari 1 Malformation, it shows the CM and also shows the syringomyelia or "syrinx" for short. (Yes syrinx is spelled that way.)
So here is what my two kids chiari looks like up close:
Picture from: http://www.chiariinstitute.com/chiari_malformation.html
References:
http://www.mayoclinic.com/health/chiari-malformation/DS00839
http://www.ninds.nih.gov/disorders/chiari/detail_chiari.htm
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