A 4-year-old boy presented with a cervical enterogenous cyst manifesting as right arm pain. Neurological examination revealed weakness of his right upper extremities and increased deep tendon reflexes. This isolated, cervical intraspinal enterogenous cyst was treated successfully by posterior laminectomy, intradural exploration, and subtotal resection. He made a good recovery without additional neurological deficits. A brief overview is given of this rare cyst, together with their clinical, pathological, radiological features and associated findings.