Thrombocytopenia occurs in up to a third of preterm neonates admitted to intensive care units. In these babies, thrombocytopenia typically presents in one of two patterns: early-onset thrombocytopenia occurring within 72 h of birth and late-onset thrombocytopenia which develops after 72 h. Early-onset thrombocytopenia is most commonly caused by disorders associated with placental insufficiency (e.g. maternal hypertension), is mild-moderate, self-limiting and requires no treatment; it is caused by reduced platelet production. Late-onset thrombocytopenia is usually due to bacterial sepsis or necrotising enterocolitis; it is often severe (platelets <50 x 10(9)/l), prolonged and requires treatment with platelet transfusions. In term babies, neonatal thrombocytopenia is usually severe and most commonly caused by bacterial sepsis, perinatal asphyxia or neonatal alloimmune thrombocytopenia. There is a lack of evidence-based guidelines for treatment of neonatal thrombocytopenia. The most important future developments will depend upon studies aimed at determining optimal platelet transfusion schedules for term and preterm neonates.