Background:Craniovertebral junction (CVJ) abnormalities constitute an important group of treatable neurological disorders with diagnostic dilemma. Their precise diagnosis, identification of probable etiology, and pretreatment evaluation significantly affects prognosis and quality of life of patients.Aims:The study was to classify various craniovertebral junction disorders according to their etiology and to define the importance of precise diagnosis for pretreatment evaluation with multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI).Materials and Methods:This is a prospective observational study of 62 patients referred to our department between October 2012 and September 2014. All patients suspected to have a craniovertebral junction disorder were included in the study, from all age groups and both genders. Detailed clinical history was taken. Radiographs of cervical spine were collected if available. All patients were subjected to MDCT and/or MRI.Results:In our study of 62 patients; 39 were males and 23 were females, with male to female ratio of 1.6:1. Most common age group was 2nd -3rd decade (19 patients, 30.64%). Developmental anomalies (33 patients, 53.22%) were the most common etiology group followed by traumatic (10 patients, 16.12%), degenerative (eight patients, 12.90%), infective (four patients, 6.45%), inflammatory and neoplastic (three patients each, 4.8%), and no cause found in one patient.Conclusions:CVJ abnormalities constitute an important group of treatable neurological disorders, especially in certain ethnic groups and are approached with much caution by clinicians. Thus, it is essential that radiologists should be able to make a precise diagnosis of craniovertebral junction abnormalities, classify them into etiological group, and rule out important mimickers on MDCT and/or MRI, as this information ultimately helps determine the management of such abnormalities, prognosis, and quality of life of patients.