The endoscopic endonasal approach is emerging as a feasible alternative to the transoral route for the resection of the odontoid process, when the latter produces a compression of the brainstem and cervicomedullary junction. This type of approach has some advantages, such as excellent prevertebral exposure of the craniovertebral junction in patients with small oral cavities and the possibility to avoid the use of mouth retractors. In this article, we present a typical case of a 12-year-old female patient with a diagnosis of craniovertebral anomaly-basilar invagination into foramen magnum, suffering from an extreme posterior tilt of the odontoid process causing severe anterior compression of the cervicomedullary junction, is presented to stress the potential of this technique. Transnasal endoscopic removal of the odontoid process was performed and resolution of the ventral compression was achieved. This article demonstrates that in selected cases, a multidisciplinary team approach can help our patients.