Myelomeningocele presenting as a retromediastinal mass originating from the midline defect through the fused T-4, T-5, and T-6 VBs is described. An 11-year-old boy with a completely normal systemic and neurological examination suffering pulmonary problems such as effort dyspnea and severe kyphosis was evaluated and an anterior thoracic myelomeningocele was diagnosed. The boy underwent surgery for excision of the meningomyelocele sac and correction of kyphoscoliosis. His year-long follow-up period as an outpatient was uneventful. A comprehensive PubMed search of the literature returned no results for an "anterior thoracic myelomeningocele" query. To the best of the authors' knowledge, this is the only case described in the literature.