Spondylolisthesis associated with neurofibromatosis is rare, and only 12 cases have been reported so far. However, only one report of grade 4 spondylolisthesis with neurofibromatosis has been reported in the literature. A 15-year-old boy with neurofibromatosis was admitted for back pain and neurological claudication. Radiograph showed grade 4 spondylolisthesis of the L5 vertebra with scalloping of the L4-L5 vertebrae. L4-L5 laminectomy, reduction, L3-S1 posterior instrumentation and fusion were performed. The reduction of the spondylisthesis was done entirely from the posterior approach using pedicle screws. Radiography at four months showed a broken S1 screw with a loss of reduction. The patient was re-operated on, to provide additional stability with pelvic fixation. He was pain-free with a good fusion at the two-year follow-up. Adequate posterior stabilisation with fusion gives good results in grade 4 spondylolisthesis associated with neurofibromatosis and dural ectasia.