The paper describes a severe form of chronic granulomatous disease with onset in the neonatal period; the case presented is a 2-month-old male infant, coming from a family in which hid older brother died with signs of the same illness. The disease is rare. This is the second report in our literature. The clinical picture showing various severity confirms the heterogenity of the disease and difference in the gene expressivity. Further investigation involving the function of granulocytes in children with recurrent infections might help us to reveal this disease characterized by disfunction of granulocytes, more frequently.