Introduction <p>Skeletal dysplasias (SD) are a rare condition; among these, the Méarbané-Dagher-Melki dysplasia is very rare and lead to early death. The case presented is a patient who was diagnosed at birth with spondylometaphyseal dysplasia. At 3 years the patient underwent occipital decompression and fusion due to spinal instability and cord compression at 4 years the diagnosis of spondylometaphyseal dysplasia Megarbane-Dagher-Melki type was made. Surgical intervention using a growth-friendly technique was performed at 11 years due to progressive kyphoscoliosis. At this time, proximal and distal anchors were inserted with allogenic bone graft, followed by 10 weeks of halo-gravity traction. The growing rods system was then completed successfully without complications. At 13 years old, the patient is the oldest known individual with this genetic mutation.</p> Discussion <p>Craniocervical anomalies and kyphoscoliosis are common in SD. Vertebral anatomy and radiographic findings challenge interpretation and surgical decision-making. In this patient, cervical MRI was performed early. The risk of neurologic injury from an unstable cervical spine exceeds that of surgical fusion. Patients with SD face high surgical risks. In this patient, a growth friendly approach was adopted. Staging the procedure minimized risks and allowed progressive correction. Early anchor placement promoted local fusion, enhancing stability, while gradual correction lowered neurological risks. Megarbane dysplasia is a fatal condition, with reported cases of affected children succumbing at a very young age. This child is the only and oldest known survivor.</p>