OBJECTIVE Our objective was to determine the neurodevelopmental outcome at 18-24 months' of corrected age (CA) in preterm infants with severe intraventricular hemorrhage (IVH). METHODS This was a retrospective cohort study of all preterm infants who were <37 weeks' gestation, had Grade 3-4 IVH, were admitted between January 2009 and December 2010 and discharged. The cohort was divided into three groups. Group 1 was defined as infants born with a birth weight (BW) less than 1000 g, group 2 was defined as infants born with a BW between 1000 and 1500 g and group 3 was defined as infants born with a BW between 1501 and 2500 g. Severe IVH was defined as the presence of grade 3-4 IVH on cranial ultrasound. Cranial ultrasound was performed in the first week of life and subsequently at weekly intervals by a radiologist. A comprehensive assessment including hearing, vision, neurological and developmental evaluation with Bayley Scales of Infant Development, Second edition was performed by the experienced researchers at 18-24 months' CA. Neurodevelopmental impairment (NDI) was defined as at the presence of one or more of the following: cerebral palsy; Mental Developmental Index score lower than 70; Psychomotor Developmental Index score lower than 70; bilateral hearing impairment; or bilateral blindness. RESULTS From January 2009 to December 2010, a total of 138 infants were diagnosed as severe IVH (grade 3-4). Of them, 74 (71.1%) infants (group 1 = 31, group 2 = 29 and group 3 = 14 infants) completed the follow-up visit and evaluated at 18-24 months' CA. Median Apgar score (p < 0.01) and resuscitation at birth (p < 0.01) were significantly different for groups 1-3. The use of catheterization, need for mechanical ventilation, need for phototherapy, retinopathy of premature and bronchopulmonary dysplasia were significantly higher in group 1 compared to groups 2 and 3 (p < 0.001, p < 0.001, p < 0.01, p < 0.01 and p = 0.014, respectively). The duration of hospitalization and mortality rates consistent with the degree of prematurity were significantly higher in group 1 compared to groups 2 and 3 (p = 0.03 and p = 0.01). Among the long-term outcomes, the rates of CP and NDI did not differ between the groups (p = 0.68 and p = 0.068). CONCLUSIONS Our results demonstrated that long-term outcomes of preterm infants did not differ between the groups classified according to the BW at two years of age. This has leaded to the conclusion that severe IVH is alone represents a significant risk factor for poor neurodevelopmental outcome in this already high-risk population.