We report a case of late vitamin K deficiency bleeding (VKDB) in a two-month-old girl who had not received prophylaxis at birth. She was exclusively breastfed without oral vitamin K supplement. She presented with catastrophic central nervous system bleeding when laboratory findings revealed mild cholestasis and coagulopathy compatible with vitamin K deficiency. She died despite intensive care and correction of coagulopathy. VKDB remains an uncommon but significant risk to infants without vitamin K prophylaxis. The current medical literature supports that intramuscular vitamin K injection given at the time of birth is the most effective and reliable means of prophylaxis. The risk of childhood cancer associated with parenteral vitamin K injection at birth has not been proven and is unlikely to be clarified in the near future. If a regimen of oral prophylaxis is adopted, the physicians and the parents must closely follow the drug administration and accept the slightly increased risk of VKDB.