The Chiari Malformation is a congenital anomaly of the craniovertebral junction and hindbrain. It is characterised by the herniation of posterior fossa structures through the foramen magnum into the cervical spinal canal. Although Chiari malformations were described at the end of the 19th century, they are still the main subject of many studies because their pathogenesis and natural course are not fully explained, asymptomatic cases are increasingly detected and accompaying pathologies pose separate problems. In this review, information about Chiari Malformations and the causes of accompaying pathologies and treatment planning will be given.