Bacteria belonging to the genus Tsukamurella are aerobic, gram-positive rods that are weakly acid-fast with no apparent branching. Infections of the Tsukamurella spp. are generally caused by the use of infected medical devices such as central venous catheters. The underreporting of these infections might be attributable to the frequent misdiagnosis of Tsukamurella infections as Corynebacterium or atypical Mycobacterium spp. infections. Therefore, when gram-positive aerobic rods are observed in the blood culture of a patient with a central venous catheter, it is important to consider Tsukamurella as one of the causative organisms. Here, we report the first case of a catheter-related blood stream infection caused by Tsukamurella inchonensis in a 3-yr-old Korean girl with underlying biliary atresia who underwent hepatoportoenterostomy.