In April 2001, a male infant (2 lbs, 13 oz [1,270 grams]) was delivered by cesarean section at 33.5 weeks' gestation and was hospitalized in a neonatal intensive care unit (NICU) because of low birthweight, prematurity, and respiratory distress. The infant had fever, tachycardia, decreased vascular perfusion, and neurologic abnormalities (e.g., suspected seizure activity) at 11 days. Cerebrospinal fluid (CSF) obtained by lumbar puncture was analyzed and revealed a white blood cell count of 32/mm3[normal = 0-0.5/mm3], red blood cell count of 27/mm3 [normal = 0], protein of 292 mg/dL [normal = 15-45 mg/dL], and glucose of 1 mg/dL [normal = 40-70 mg/dL]. Culture of CSF grew E. sakazakii. The infant was treated with intravenous antimicrobials for meningitis; however, neurologic damage was progressive, and the infant died 9 days later. Because the organism was a rare cause of neonatal meningitis, hospital personnel, in collaboration with the Tennessee Department of Health and CDC, investigated the source of infection.