© 2013 Springer Science+Business Media New York. All rights reserved. There exists a plethora of diseases affiliated with CIM and certainly more to be discovered in the future. While the final outcome of CIM may be the same, the strength of the correlation and pathophysiological mechanisms of each differs greatly, and some may be spurious associations. Thus, the need for additional genetic research and investigation of CIM continues. This chapter will discuss the conditions associated with CIM in order to potentially shed light on how the pathophysiological mechanism of one condition, no matter how remote, might lead to the development of CIM. It should be noted that many of these associations may be incidental with an asymptomatic hindbrain hernia being identified due to testing for other pathologic entities, for example, endocrinopathies.