The objective of this study was to evaluate urinary vanillylmandellic acid (VMA) as a marker for prognosis and progression of neuroblastoma. A retrospective file search of 444 patients during 33 years was performed and correlation of VMA with clinical prognostic parameters and outcome was evaluated. Event-free survivals (EFS) were 33.5% and 21% (P = .04) and overall survival (OS) were 36.6% and 25.8% (P = .1) for all patients with normal/negative and increased/positive VMA. EFS and OS were higher in VMA(-) pelvic (P = .03) and thoracic and neck (P = .04) tumors, compared to their VMA(+) counterparts. Survival was not different in patients with abdominal primaries according to VMA status. Positive urinary VMA prevalence was low in localized disease and high in disseminated disease (P < .001). In disseminated disease, 10-year EFS was higher in VMA(+) patients than VMA(-) patients (16%, 9.5%, P = .054). Two-year OS was higher in VMA-positive patients with stage 4 disease (20.6% and 7%, P = .04). The patients with VMA(+) progressive disease died later than those with VMA(-) tumors (P = .047). These results show that increased urinary VMA predicts poor outcome for patients with favorable tumor sites. Urinary VMA can be useful risk determinant in combination with other biological determinants to predict prognosis of patients with localized neck, thoracic or pelvic tumors. The relationship of positive or high urinary VMA and survival were inversely correlated in disseminated disease and in patients with progressive disease. In high-risk patients, negative spot test or normal level of urinary VMA at diagnosis might be used for identification of a group of patients with more favorable prognosis.