Background and aim Cervical canal stenosis is a rare congenital anomaly that may cause cervical myelopathy. Symptomatic congenital cervical stenosis manifests predominantly in adults. No reports have described a case in the immediate neonatal period. We here report the case of a 3-day-old neonate who presented with symptoms of cervicomedullary compression early in the neonatal period. Materials and methods The newborn was referred to our institution for investigation of hypotonia, respiratory distress and apnea spells. Shewas born by elective caesarean section at the 37thweek of gestation to nonconsanguineous parents. Apgar scoreswere 8 at 1 min and 8 at 5 min. The initial physical examination disclosedmarked neck and upper-limb hypotonia unassociated with no dysmorphisms. A thorough investigation of metabolic and genetic diseases possibly associated with hypotoniawas non diagnostic. Because the apneic eventswere related to meals, the patient underwent tests for evaluation of gastroesophageal reflux. Esophagogastroduodenoscopy showing esophagitis and hiatal hernia confirmed the diagnosis and treatment was started with the proton pump inhibitor lansoprazole. Despite treatment, the apnea spells progressively worsened. A polysomnogram documented frequent, prolonged episodes of central apnea associated or unassociatedwithmeals. Because no other cause could be found to explain why apnea and hypotonia persisted, a magnetic resonance imaging (MRI) scan of the encephalus and craniocervical junction was obtained and disclosed a foramen magnumnarrowing compressing the upper spinal cord but no other abnormality.