BACKGROUND: Thoracic intervertebral disk herniation is an uncommon entity. Acute presentation of this entity in the pediatric population is exceptionally rare. Given the infrequent prevalence, accurate diagnosis of acute symptomatic thoracic disk herniation can be difficult. PURPOSE: The purpose of this article is to describe the presentation of acute nontraumatic thoracic disk herniation in a pediatric patient and the diagnostic workup and surgical management of this rare entity. STUDY DESIGN: This is a case report regarding a pediatric patient with nontraumatic acutely symptomatic thoracic disk herniation. The order of diagnostic studies and method of surgical decompression used are described. Progressive follow-up neurological improvement is detailed in the report. METHODS: A 16-year-old adolescent girl with acutely symptomatic paramedian disk herniation at T10 to T11 underwent left thoracotomy followed by microdiscectomy. Decompression of T10 to T11 was followed by fusion of T10 to T11 with rib strut graft. RESULTS: Postoperatively, the patient recovered near-complete resolution of bilateral lower extremity paralysis and dysesthesias. CONCLUSIONS: Thoracic intervertebral disk herniation is a rare phenomenon. It is a particularly uncommon entity in the pediatric population. As such, the diagnosis and management of thoracic disk herniation can be a considerable challenge. As illustrated in our case report, the clinician's focus should not exclusively rest on lumbar disk pathology as the etiology for such rapidly evolving neuromuscular deficits. Thoracic disk herniation must be included in the differential diagnosis, and appropriate diagnostic workup should be instituted in an expeditious manner. Plain radiographic studies may not delineate the causative factor of pathology, and emergent magnetic resonance imaging can aid in obtaining a timely diagnosis. Early intervention and decompression have been shown to significantly improve functional recovery.