This report describes a novel method of repair for a large thoracolumbar myelomeningocele with an associated lumbar kyphosis in the neonate. A Caucasian male child was born at term with an antenatal diagnosis of hydrocephalus and spina bifida. Lumbar spine X-ray showed a significant kyphosis at L2-L3 level. Kyphectomy was performed and a cervical plate was used to reduce the gibbus deformity and maintain a rigid construct. Local rotation flaps were elevated and advanced to cover the defect. Wound was well healed at 3-month follow-up and the patient remained shunt-free at 1 year. To our knowledge, this is the first description of myelomeningocele repair with kyphectomy and posterior fixation in a neonate.