Neurofibrosarcoma is rare in the pediatric age group. A malignant tumor of the sacrum presents significant challenges, especially if the goals are to resect with wide and clean surgical margins and to achieve acceptable functional outcomes. The authors report a case of this rare tumor affecting the sacrum and sacral nerve roots of a 7-year-old girl and review the role of total hemisacrectomy sparing the contralateral sacral nerve roots and lumbopelvic reconstruction in the treatment of this disease. This patient is, to the best of the authors' knowledge, the youngest to be treated in this manner.
Keywords: MPNST = malignant peripheral nerve sheath tumor; UTI = urinary tract infection; hemisacrectomy; neurofibrosarcoma; oncology; pediatric spine; sacral tumor; sacrectomy.