An association between early-onset neonatal Group B streptococcal (GBS) sepsis and subsequent diagnosis of late-onset diaphragmatic hernia has been reported. We report one such case and review the literature. The index case involved a male preterm neonate in whom late-onset right diaphragmatic was diagnosed on day 21 following early-onset GBS sepsis. Our literature review identified 40 cases of such an association. The typical description involves near term male neonates, who improved from an early-onset GBS sepsis only to have a secondary respiratory deterioration leading to the diagnosis of late-onset right diaphragmatic hernia. With surgical repair, this condition has an excellent survival rate. In conclusion, the pathophysiological mechanisms underlying the association remain to be elucidated. Awareness of the associated pleural effusion is important to avoid inadvertent organ injury due to chest drains.