<p>Lateral meningoceles are defined as protrusions of the arachnoid and dura mater through the intra- and intervertebral foramina of the spine. Dural dysplasia, such as dural ectasia, contributes to their occurrence. Lateral meningocele syndrome (LMS) is an extremely rare genetic connective tissue disorder. A 3-year-old boy began to frequently slip and fall. At 5 years of age, he developed progressive intermittent claudication with radiating pain along the lateral aspect of the right thigh. Physical examination revealed bilateral blepharophimosis, downslanting palpebral fissures, hypertelorism, micrognathia, retrognathia, low-set ears, and a right-sided paraspinal protrusion. Spinal magnetic resonance imaging (MRI) at 6 years of age demonstrated multiple lateral meningoceles of the thoracic and lumbar spine with posteriorly scalloped vertebral bodies. Computed tomography (CT) revealed severe scalloping of vertebral bodies and pedicles from T8 to L4 with widening of the spinal canal. Two cyst-subarachnoid (C-S) shunts were placed, at the superior aspect of the meningocele (T8) and the inferior aspect (L5–S1). His symptoms improved immediately, and the meningocele gradually regressed postoperatively. LMS is a rare genetic disorder associated with craniofacial anomalies, developmental delay, intellectual disability, hypotonia, and decreased muscle mass. Although C-S shunting successfully reduced intracystic pressure in this case, long-term neurological and radiological follow-up is required to determine whether shunting can halt the progression of lateral meningoceles.</p>