Despite the remarkable amount of clinical and biologic information that has been cumulated on neuroblastoma, particularly in the last two decades, this embryonal tumor of early childhood remains one of the major challenges of pediatric oncology. It is now clear that the term encompasses at least two entities characterized by different features and outcome. The favorable entity includes patients with localized disease, infants and stage IV-s. The unfavorable entity refers to patients older than one year at diagnosis with disseminated disease. However, in both entities exceptions exploiting a clinical course different from expected do occur. Molecular biology is presently suggesting that specific genomic alterations may predict unfavorable events. Patients evaluation should thus include the study of biologic features since they may provide us with strongly predictive clues. In this article the Authors describe the main clinical features of the neoplasia and focus on some of its more peculiar patterns. They also refer on the criteria that the International Neuroblastoma Staging System (INSS) has recently designed regarding diagnosis, evaluation of disease extent, and response to therapy, in order to improve the cooperations and the understanding among the major Cooperative Groups. Finally, the treatment outlines of the main forms of clinical presentations are described.