Introduction. Intramedullary tumors in craniocervical junction are divided into descending tumors of brainstem and ascending, in cranial direction, spinal tumors in cervical area. In both group most common are low grade gliomas with predominant part of astrocytoma fibryllare WHO II. Main symptoms develop because of high intracranial pressure or spinal cord syndromes. Additionally occurs torticollis, balance disturbances (problems with ambulation, ataxia), lower cranial nerves deficits or paresis. Variety of presenting symptoms impedes a proper diagnose and has an influence on results of treatment. Material and method. To reach the aim of the trial the analyses of literature and empiric examination was used. We retrospectively analyzed 9 cases of children treated on tumors of craniocervical junction in Neurosurgery Department of Children’s University Hospital in Krakow. There are 5 girls and 4 boys in the age between 2 and 16 years old. It has been analyzed: main symptoms, CT and MRI imaging, surgical techniques, results of treatment and patients quality of life after therapy. The analysis of literature and electronic bases was also conducted. Outcomes. Among many symptoms, in this analysis most common signs were: headache, vomiting, balance disturbances, ataxia, paresis, torticollis and diplopia or deficits in visual field. All patients underwent CT and MRI scanning of head and neck. Additionally MRI scans of thorax, abdomen and pelvis was used. Surgical techniques included: craniotomy, laminotomy, subtotal and total resection of tumor followed by posterior stabilization. In six patients a complete remission over 5 years was achieved. Three other patients are still during treatment and observation. Conclusion. Treatment of choice in tumors of craniocervical junction is total resection of neoplastic area, with good results in long duration of remission. On the other hand total resection of tumor in localization like brainstem and cervical spinal cord brings the risk of neurological disturbances. Controversial, especially due to age of child, is use of posterior stabilization.