We report herein the case of a 56-year-old woman with a neuroblastoma associated with a high serum level of carbohydrate antigen, CA125. The patient presented with massive ascites and a firm mass in her upper abdomen for which a laparotomy was performed. However, a recurrent tumor was found 6 months later and she died of the disease within 1 year of surgery despite several courses of adjuvant chemotherapy. Neuroblastoma rarely occurs in adults, and the features of 58 adult cases described in the world literature is summarized following the presentation of the clinical data on this case. The distribution of primary sites in adults is dispersed compared to that seen in pediatric cases, while the natural history of the disease in adults may be longer and less sensitive to chemotherapy than in children. The survival rate of adults with this disease is poor. We conclude that aggressive surgical intervention combined with appropriate chemotherapy protocols as applied in children should be performed in an attempt to achieve complete remission and improve the survival rate of adults with neuroblastoma.