Chiari I malformation is a well-described entity characterized by hindbrain herniation through the foramen magnum. Although the exact origin of congenital Chiari I malformation is unknown, it appears to be caused by a mismatch between the volume of the posterior fossa neural elements and the posterior fossa cranial content. Several theories have been proposed to describe the resultant pathophysiology of this mismatch. It is clear, however, that abnormal cerebrospinal fluid flow and velocity play a role in the symptoms and signs associated with this disorder. The authors will review the pathophysiology, clinical presentation, and treatment options for patients with Chiari I malformation.