Purpose: To evaluate the value of random urine VMA at diagnosis as a surrogate marker in monitoring the tumor response and predicting the outcome in patients with neuroblastoma. Methods: Medical records of 105 patients who were newly diagnosed with neuroblastoma at Samsung Medical Center between June 2014 and August 2017 were reviewed. Clinical association with other prognostic factors including age at diagnosis, stage, pathologic subtype, MYCN amplification, and other cytogenetic aberrations were analyzed. In addition, the significance of random urine VMA in predicting the outcome and tumor response was also evaluated. Results: Median value of random urine VMA at diagnosis was 27.0 mg/g creatinine (range 1.7∼＞600). Abdominal primary site ( P=0.029), male ( P=0.016), advanced stage ( P0.001), less differentiated pathology ( P=0.010), 11q deletion ( P=0.001) and high-risk tumor ( P0.001) were associated with a higher VMA level at diagnosis. VMA level decreased during chemotherapy (38.2%, 26.3%, and 13.0% of VMA at diagnosis after 3, 6, and 9 cycles of chemotherapy, respectively). A higher VMA level at diagnosis was associated with worse overall survival in high-risk patients with borderline significance (40.0±29.7 vs 95.0±4.9%, P=0.070). However, a slower reduction in VMA level during chemotherapy was not associated with a worse outcome. Conclusion: A higher random urine VMA was associated with high-risk features at diagnosis. Urine VMA can be used as easy and useful marker in monitoring the tumor response during chemotherapy.