The discovery of a neutropenia is a common problem in pediatric population. Some children with neutropenia have an uncomplicated clinical course and require no treatment. However, some patients are more severely affected and present a management challenge for the pediatrician and for the hematologist. The clinical approach to these children is directed primarily to understand the etiology of neutropenia in order to define a correct therapeutic behaviour, such as glucocorticoids, intravenous immunoglobulins or, more recently, rhG-CSF. In our article we have reviewed the etiology, the clinical course and the therapeutic options of constitutional and acquired neutropenias.