Neurenteric cysts are rare congenital spinal masses that result from the dysgenesis of the endoderm tissue during development. We report a 4-year-old girl who presented with an insidious onset of lower limb paraparesis. An MRI scan revealed a cervicothoracic intradural extramedullary neurenteric cyst at the thoracic T1/T2 level, with marked spinal cord compression. No associated spinal dysraphism was noted. The patient underwent laminotomy and excision of the cyst. She recovered her neurological functions completely post-operatively, and at her six-month follow-up she was asymptomatic without any neurological deficits. We will discuss the pathogenesis, clinical presentation, and neuroradiological findings. We emphasize the value of early surgical intervention and long-term follow-up when this type of lesion is only partially excised.