 Inherited thrombocytopenias are a heterogeneous group of disorders characterized by a reduced number of blood platelets and a consequent bleeding tendency that ranges from mild to life-threatening. Thrombocytopenia is caused by genetic mutations and therefore is present throughout patients life and can be transmitted to the progeny. Some patients with severely reduced platelet count present spontaneous bleedings, which represent a major clinical problem: in fact, bleeding diathesis exposes these subjects to the risk of severe hemorrhages, affects their quality of life and often requires hospitalization andor transfusions. Conversely, other patients with inherited thrombocytopenias have absent or mild spontaneous bleeding tendency. However, even these patients are at risk of major bleedings on the occasion of surgery or other invasive procedures. Therefore, the potential for hemorrhages on the occasion of invasive procedures represent a clinical problem for all patients affected by inherited thrombocytopenias. 