We examined longitudinal neurodevelopmental outcomes in a series of infants with microcephaly. Retrospective review identified neonatal intensive care unit follow-up clinic patients with a diagnostic code of microcephaly, verified by head circumference less than the fifth precentile (WHO growth curves). Data were collected regarding clinical history and developmental assessments by Capute Scales and gross motor age equivalent. Developmental Quotient (DQ) was age adjusted up until 2 years for preterm infants. Twenty-two infants had microcephaly. At latest follow-up (3-66 months, mean 27.2), 73% had delay (DQ < 70) in ≥1 area of development: gross motor 65% (mean DQ 56.8), visual-motor 59% (mean DQ 62.7), and language 59% (mean DQ 65.9). In this sample, postnatal onset and diagnosis of epilepsy were associated with lower DQs. We conclude that infants with microcephaly are at significant risk for delay across all aspects of development and for long-term disability. Postnatal etiologies of microcephaly and infants with comorbid epilepsy had worse outcomes.