SUMMARY: Lactococcus lactis is a gram-positive coccus that is nonpathogenic in humans. Herein, we present the case of a 1-year-old boy with Down syndrome and Hirschprung's disease (HD) who developed a catheter-related bloodstream infection with L. lactis after gastrointestinal surgery. The patient had been hospitalized in the pediatric surgery unit from birth because of HD, and had undergone the Duhamel-Martin procedure which caused recurrent diarrhea episodes and feeding intolerance. On the infant's 430th day of life, he had an episode of gastroenteritis and feeding intolerance. Because of clinical suspiction of sepsis, blood cultures were taken both from the central venous catheter and peripheral vein, and evidence of a growing microorganism was detected in 2 different central venous catheter blood cultures taken 2 days apart. The colonies were then identified by both the Vitek 2 and Vitek MS systems (bioM áerieux, Marseille, France) as L. lactis spp. lactis. The central venous catheter could not be removed because of the absence of a peripheral venous line, and the patient was subsequently successfully treated with vancomycin. Therefore, although Lactococcus species is generally thought to be nonpathogenic, it should still be kept in mind as a potential pathogen in infants.Catheter-related bloodstream infections (CR-BSIs) are an important source of morbidity and mortality worldwide. Lactococcus spp. are not recognized as important pathogens in humans, but the current literature provides evidence that they can cause infection, particularly in immunocompromised hosts (1). In this case study, we describe the second known case of CR-BSI caused by L. Lactis in an infant.A 1-year-old boy with Down syndrome (DS) had been hospitalized in the pediatric surgical care unit since birth. Because of vomiting, abdominal distension, and meconium ileus, rectal biopsy was performed 14 days after birth, revealing the absence of ganglion cells, he was thus diagnosed with Hirschprung's disease (HD). An ileostomy was performed on his 30th day of life. When the patient was 1-year old, the definitive Duhamel-Martin procedure was performed. The patient could not be discharged because he required total parenteral nutritional support from a central venous line via an 8-cm Triple-Lumen 5.5-Fr ARROW Catheter inserted into the femoral vein.On the patient's 430th day of life, he had an episode of gastroenteritis along with feeding intolerance, erosive diaper dermatitis, and a worsening clinical condition.He was also hypotensive and hypotonic subfebrile rise in temperature. No pathogenic microorganisms were found on stool. The laboratory results showed a white blood cell count of 14,800/mm 3 and C-reactive protein levels of 29.3 mg/L (0-5 mg/L). Furthermore, the infant's urinalysis was normal, his urine culture remained sterile and he had no signs of pneumonia.Because of the clinical suspiction of sepsis, blood cultures were taken both from the central venous catheter and peripheral vein. Fourteen hours later, the BacT/Alert device (bioM áe...