Craniocervical junction tumors are rare. Tumors of this region may originate from soft tissues, neural and bone structures. The most common benign tumors in adults at the craniocervical junction are aneurysmal bone cysts, osteoblastomas, eosinophilic granulomas, and solitary plasmacytomas. The most common malignant neoplasms are chordomas, chondrosarcomas, osteosarcomas, and metastatic tumors. The most common primary benign tumors of the craniocervical junction in children are osteoid osteomas, osteoblastomas, and aneurysmal bone cysts. The most frequent malign tumors of this region is chordomas. The symptoms are variable due to the localisation, histological type and size of tumor. It usually occurs due to compression or traction of neurons. Various imaging modalities are used for diagnosis, including magnetic resonance imaging, computed tomography, dynamic and vascular imaging. Treatment modalities of craniocervical junction tumors are diversed by histological diagnosis, tumor location and neurological status. Surgical approach is difficult and risky due to the presence of neurovascular vital structures localized in this deep and narrow anatomical structure of the region. Advanced radiotherapy options can also be an option adjucted to surgery.