Background: Basilar invagination (BI) is an uncommon clinical condition of the craniocervical junction (CCJ). Surgical management depends on 2 factors: mobility and reducibility; in cases of irreducible dislocation or persistent compression, odontoidectomy should be considered.

Case discussion: We present the case of a 13-year-old boy with severe BI, causing cervical myelopathy with progressive gait disorder. The patient underwent cervical traction followed by posterior decompression and occipitocervical fusion. Postoperatively, symptoms initially improved, until new neurological deterioraton set in 4 months later. Follow-up neuroimaging showed compression of the bulbo-medullary junction, with severe brainstem kinking and appearance of a cervical syrinx. Secondary surgery via an endoscopic endonasal approach (EEA) was deemed necessary to relieve the compression. Postoperative course was unremarkable, with steady clinical improvement and a return to independent activities of daily living within 6 months.

Literature Review: A systematic literature review indicated that EEA conserves the palate and oropharynx mucosae, thus causing less airway and swallowing complications than the transoral approach.

Conclusion: In selected cases with persistent anterior compression, odontoidectomy on EEA is a safe, effective and valid alternative for managing CCJ pathology.