Central venous catheterization has been frequently used in pediatric patients in wide variety of conditions. Several authors have described about various complication of central venous catheterization in pediatric patients and reported complication rates of 3% to 6%. Knotting of guidewire as a complication of central venous catheterization has been reported in adult patients, but knotting of guidewire has not been reported in infants. Therefore we report an infant who experienced a knotting of guidewire during internal jugular vein catheterization which was successfully removed without any adverse events. We suggest that knot formation is possible in infants and there should be a high suspicion if resistance is felt at the time of catheter advancement.