Introduction: Acute bilirubin encephalopathy (ABE) is associated with long-term sequelae (kernicterus). It continues to be a significant issue in our region of Nigeria, accounting for much morbidity and mortality. Herein we report the outcome of neonates with ABE seen at our centre. Methodology: We established a surveillance of children who had ABE and returned to follow-up from prospective cases of ABE (2012-2014). ABE was diagnosed based on a bilirubin-induced neurologic dysfunction score of ≥ 1. Kernicterus was subsequently established based on a history of developmental delays, hearing impairments and abnormal physical and neurologic examinations at follow-up age ≥3 months. Result: Five hundred fifty-one neonates had hyperbilirubinaemia of whom 104 (18.8%) had ABE. Mean transcutaneous bilirubin using the Ingram icterometer was 18.3 mg/dl ± SD 1.9 [(12.5-19.1), total serum bilirubin of 18.1 ± 10.9] (range: 10.3-64 mg/dl). Sixty-five infants returned for follow-up (41 males and 24 females); mean age 9 months (22 days to 17 months). Most (58 of 65; 89.2%) had abnormal neurological findings and 15 (25.9%) had probable kernicterus. Conclusion: There is a critical need for a National Kernicterus Registry to document all cases of kernicterus and formulate an effective treatment and prevention policy.