Primitive intraneural synovial sarcomas are rare in children. The authors report the case of a 7-year-old girl affected by intraneural synovial sarcoma of a lumbar nerve root, the first such lesion in this location described in a child. The lesion mimicked a schwannoma clinically and radiologically. There was long-lasting leg pain in a radicular distribution, and a well-demarcated intraneural tumor was seen on MRI. On this basis, the first resection was conservative. However, histological examination documented a classic biphasic synovial sarcoma, which was confirmed by immunohistochemistry. After radical resection and adjuvant treatment, complete disease control was achieved and verified at 5-year follow-up. This case strongly suggests that early diagnosis and a multidisciplinary approach to this unusual spinal lesion are essential to achieving a better prognosis.