The congenital and acquired deformities of the craniovertebral junction (CVJ), such as basilar invagination, basilar impression, or platybasia, can present in the form of slowly progressive or acute neurologic deterioration. In many cases, an insidious headache is the only symptom and can be a diagnostic challenge for the neurologist. Proper imaging studies as well as recognizing often associated neurologic or systemic conditions are required for early diagnosis and effective therapy. In the current report, the primary focus will be on clinical aspects of these CVJ abnormalities; the pathologic and radiologic aspects, such as developmental and pathophysiologic background or radiographic analysis, will be discussed briefly, confined to clinically relevant data.