Burkholderia cepacia causes sepsis in neonates who are immunocompromised or exposed via nosocomial transmission. We report a case of B. cepacia sepsis in a previously healthy 5-week-old male originally treated for bacterial pneumonia per chest X-ray findings and 3 days of fevers. Regardless of appropriate antibiotics and an initial negative blood culture, he developed severe hypoglycemia, circulatory collapse with disseminated intravascular coagulopathy, and expired. A second blood culture taken following transfer to the intensive care unit resulted positive for B. cepacia postmortem. Review of the newborn screen and family history was otherwise normal. Subsequent postmortem autopsy showed multifocal bilateral pneumonia with necrotizing granulomatous and suppurative portions of lung tissue. Additionally, there was a prominent cavitary lesion 2.5 cm in the right lower lobe with branching and septate fungal hyphae. Stellate microabscesses with granulomas were present in the liver and spleen. These findings plus B. cepacia bacteremia are highly suggestive of an immunocompromised status. Review of the literature shows that its presence has been associated with chronic granulomatous disease. Therefore, in a persistently febrile infant not responding to antibiotics for common microbes causing community-acquired pneumonia, immunodeficiency workup should ensue in addition to respective testing for chronic granulomatous disease especially if B. cepacia culture-positive as it is strongly associated with neutrophil dysfunction.