Introduction <p>Spondyloepiphyseal dysplasias (SED) are rare skeletal disorders involving the spine and growth plates. This study looks at our surgical experience of managing the cranio-cervical junction and cervical spine in children with SED, emphasizing the importance of early recognition of cervical instability.</p> Materials and methods <p>This retrospective, single-centre study identified all pediatric patients with SED and cervical involvement who underwent cranio-cervical or cervical spine surgery at our institution between 2007 and 2022, with a follow-up period of at least 24 months. We collected data on the patients’ clinical and radiological characteristics, surgical procedures, perioperative complications, and outcomes.</p> Results <p>Cervical instability was detected in six out of eight pediatric patients in our cohort. Surgical treatment involved decompression and/or stabilization, with an average of 3.29 (range 2–4). Levels fused, performed at an average age of 7.7 years. At a mean follow-up period of 5.5 years, most patients showed stable or improved neurological and radiological outcomes. Four complications occurred, including one instance requiring reoperation.</p> Conclusions <p>Our study shows that cervical instability is common in pediatric patients with SED, even asymptomatic ones. This emphasizes the importance of early screening and intervention. The study also demonstrates the effectiveness and relative safety of hook-based posterior arthrodesis, despite the anatomical challenges involved.</p>