Objectives-To evaluate the developmental correlates of microcephaly evident at birth and at 2 years in a cohort born at extremely low gestational age.Methods-We assessed development and motor function at 2 years of 958 children born before the 28th week of gestation, comparing those who had microcephaly at birth or 2 years with children with normal head circumference while considering the contribution of neonatal cranial ultrasound lesions.Results-A total of 11% of infants in our sample had microcephaly at 2 years. Microcephaly at 2 years, but not at birth, predicts severe motor and cognitive impairments at 2 years. A total of 71% of children with congenital microcephaly had a normal head circumference at 2 years and had neurodevelopmental outcomes comparable with those with normal head circumference at birth and 2 years. Among children with microcephaly at 2 years, more than half had a Mental Developmental Index <70, and nearly a third had cerebral palsy. The risks were increased if the child also had cerebral white matter damage on a cranial ultrasound scan obtained 2 years previously.Conclusion-Among extremely low gestational age newborns, microcephaly at 2 years, but not at birth, is associated with motor and cognitive impairment at age 2.Ahead circumference more than 2 standard deviations (SD) below the mean for age defines microcephaly, an indicator of reduced brain volume,1 and a correlate of cognitive and motor dysfunctions.2 -5 Compared with infants born at term, low birth weight and extremely low gestational age newborns (ELGANs) are at increased risk of having microcephaly at birth (congenital microcephaly), as well as subnormal head size evident later in childhood. 2 ,3 ,6 This