Background Craniovertebral anomalies assessment is challenging and reaching the accurate diagnosis is the corner stone for appropriate management. Objective: The aim of the study was to demonstrate various imaging features of the developmental anomalies affecting cranio-cervical junction (CCJ) by using CT scan and MRI scans. Patients and methods: In the Radiology Departments in Mansoura University and Mansoura University Children^'s Hospital, 30 patients (16 males and 14 females) participated in this study at different ages with history of CCJ abnormalities by at least one imaging modality. Those patients underwent careful examination of the previous imaging scan. Some of the patients were fully diagnosed using MRI only or CT scan. Analysis was performed on the MDCT and MRI examination. Results: Arnold Chiari malformation (ACM) type 1 was the most common diagnosis followed by ACM type 2, tonsillar herniation in 3 cases, MPS, achondroplasia in 2 cases while split atlas, platypesia, Down syndrome and Os odenoottidum in 1 case each. Conclusion: Careful radiological examination of the CCJ and understanding of its complicated anatomy is required in order to reach the proper diagnosis of its lesions.