To determine the characteristics and outcomes of pulmonary arterial hypertension (PAH) in extremely low birth weight (ELBW) infants. A retrospective case-control study of all ELBW infants admitted to a level III neonatal intensive care unit (NICU) between January 1, 2003 and December 31, 2010. During the study period, 450 ELBW infants were admitted. 6.4% (29/450) were diagnosed with PAH and were matched to 26 controls. The mean gestational age of infants with PAH and their controls were similar [24.5 ± 1.3 vs. 24.9 ± 1.8 weeks (P=0.26)]; however the cases were smaller at birth than were controls [640.7 ± 119.5 vs. 727.0 ± 184.5 g (P=0.04)]. The diagnosis of PAH was made at a mean postnatal age of 131.8 ± 53.7 days. Infants with PAH had a higher rate of intrauterine exposure to illicit maternal drug use [12/29 (41%) vs. 1/25 (4%); P=0.001], a longer duration of initial mechanical ventilation [74.9 ± 28.3 vs. 59.1 ± 27.8 days; P=0.04)], a higher incidence of severe BPD [23/29 (79%) vs. 13/26 (50%); P=0.02], and a greater NICU mortality rate [12/29 (41%) vs. 4/26 (15%); P=0.04]. PAH in ELBW infants is associated with maternal illicit drug use in pregnancy, longer exposure to mechanical ventilation, severe bronchopulmonary dysplasia and a significant increase in early mortality.