Neuroblastoma is a cancer of the sympathetic nervous system. It is the most common tumor in the first year of life and the most common solid extra-cranial cancer in childhood. Neuroblastoma represents an enigmatic disease, in that some cases, especially in infants, tend to spontaneously regress, even in advanced states, while many patients are refractary to any therapeutical approach and show an inexorable clinical course. As most of the other solid cancers in children, the distribution of Neuroblastoma risk worldwide is only partly known. In Western countries its incidence varies between 7 and 16 cases per million children and it tends to decrease increasing the age. Besides, survival of patients affected by such disease, in relation with clinical and biological characteristics, have been extensively investigated only in recent years, thanks to the making of large cohoperative mulit-center research groups, while survival rates at the population are poorly investigated. The risk of developing this cancer has been related to some environmental and parental factors, but most of its etiology still remains unknown. The first part of this monograph collects the more recent information about the Descriptive Epidemiology of Neuroblastoma, providing a picture of its geographical distribution worldwide and describing the related time trend. The second part (Analytical Epidemiology) illustrates the knowledge and more recent theories about the etiology of this disease, by an extensive review of the scientific literature.