The object of this study was to describe the outcomes of children with infantile spasms resulting from perinatal stroke. We used International Classification of Diseases, Ninth Revision (ICD-9) searches of billing records to identify 110 children with infantile spasms examined at our hospital from 1998 through 2005. Five of the 99 with symptomatic spasms (5%) had perinatal stroke. An additional five children with spasms caused by perinatal stroke were identified from pediatric stroke clinic records. Seven of the 10 children with spasms due to perinatal stroke presented with stroke as neonates. Three initially appeared healthy but were diagnosed with "presumed perinatal stroke" after radiographic imaging for their spasms evaluation. Median age at last follow-up was 6.3 years: 9 (90%) had epilepsy, 8 (80%) manifested cognitive impairment, and all (100%) had cerebral palsy. The three children who had delayed presentation of "presumed perinatal stroke" had better epilepsy and cognitive outcomes than the seven with neonatal presentation (P = 0.03). Perinatal stroke accounts for 5% of symptomatic spasms and results in high rates of chronic disability similar to those observed with other types of symptomatic spasms. However, a subgroup of children with spasms caused by delayed presentation of "presumed perinatal stroke" appears to have better epilepsy and cognitive outcomes.