Introduction: Hypoplastic Left Heart Syndrome (HLHS) is a critical congenital cardiac malformation occuring in 1–2 per 10? 000 live births, defined by anatomic and functional inadequacy of the left side of the heart with non-viability of the left ventricle to perform systemic perfusion, HLHS typically presents in the early neonatal period following ductal closure, without timely intervention, rapid cardiovascular collapse is expected. Late presentation beyond the neonatal period remains rare but possible. Since the introduction of the Norwood procedure along with advances in prenatal diagnosis,neonatal care, surgical techniques, survival outcomes have improved significantly . HLHS can be diagnosed prenatally by fetal echocardiography . Case presentation: We report an unusual case of HLHS presenting at one month of age to the out-patient clinic with features of heart failure, diagnosing HLHS, later succumb to death. This term baby with good birth weight and Apgars did not exhibit typical early features and was clinically stable postnatally at discharge, with normal pre-post-ductal oxygen saturations,peripheral pulses and blood pressure. He was followed up for R/dysplastic kidney and a soft, non-specific cardiac murmur.Echocardiogram showed critical aortic coarctation, with hypoplastic arch proximal to isthmus, severe mitral stenosis and poorly formed left ventricle. Conclusion: Late presentation of HLHS is rare but possible. Atypical ductal behaviour, collateral circulation, or partial left heart function may temporarily maintain stability beyond neonatal period. Late-presenting HLHS has a high risk of sudden deterioration, emphasizing the importance of early detection, especially in cyanotic infants with poor weight gain or unexplained heart failure.