Anterior myelomeningocele manifesting as a posterior mediastinal mass has rarely been described. These anomalies, characterized by protrusion of meninges and spinal cord through open vertebral arches, manifest with devastating spinal or neurologic problems. This report describes the case of a young woman with a long-term history of right upper abdominal pain and low-grade fever but no associated neurologic or spinal defects. Relevant investigations showed a posterior mediastinal mass in the right paraspinal region. Through a right posterolateral thoracotomy, complete excision of the growth with preservation of major surrounding structures was performed. Histopathologic examination revealed myelomeningocele. This report discusses a rare case of anterior myelomeningocele at the thoracic level without neurospinal involvement.