Background: Whilst vertical transmission of Zika virus (ZIKV) is established as the cause of congenital Zika syndrome (CZS), knowledge of this emerging disease remains incomplete. Aim: To characterise the clinical, radiological and neurodevelopmental features of children antenatally exposed to ZIKV and/or presenting with suspected CZS in Jamaica, as part of the larger, international ZIKAction Paediatric Registry. Methods: This retrospective observational study (disease/exposure hospital-based registry) included children cared for at public hospitals in the Greater Kingston Metropolitan Area, Jamaica if they had exposure to ZIKV in utero, laboratory confirmation of congenital ZIKV, or met the ZIKAction’s Registry definition of suspected CZS. Maternal, perinatal and child data were extracted from hospital records and descriptive analyses conducted. Head circumference (HC) Z-scores were calculated using the Intergrowth-21st reference standards. Results: Of 53 participants, 20 (37.7%) were male. One neonate had laboratory-confirmed ZIKV, 6 (11.3%) mothers had laboratory-confirmed ZIKV, and 12 (22.6%) mothers had ZIKV-compatible symptoms in pregnancy without laboratory confirmation. Thirty (56.6%) children had congenital microcephaly (HC Z-score >-2) and 14 had severe microcephaly (HC Z-score >-3). Mean (SD) birth HC Z-score was −3.24 cm (1.0). Twenty (37.8%) infants had craniofacial disproportion and 3 (5.7%) had arthrogryposis. Among participants with evaluations, 42.4% (14/33), 43.8% (7/16), and 72.7% (24/30) had abnormal ophthalmic, audiological and neuroimaging findings respectively; 19/34 (55.8%) had developmental delay. There was one death. Conclusion: The microcephaly, physical features of CZS and adverse neurodevelopmental outcome in these children underscores the increased need for health resources and social support as they grow up.