Late-onset Vitamin K deficiency bleeding is a rare disease that develops as a manifestation of Vitamin K deficiency in children from 8 days to 6 months of age with an incidence of 4.4 to 72 cases per 100,000 newborns in countries where prophylaxis with Vitamin K1 preparations is not carried out and of 0 to 3 cases per 100,000 in countries where prophylaxis is carried out. In Russia the prophylaxis is carried out using menadione sodium bisulfite, a Vitamin K3 preparation, the effectiveness of which is questionable. One of the specific manifestations of the late-onset Vitamin K deficiency bleeding is intracranial hemorrhage (ICH), which occurs in more than half of all cases of the disease. This disease has not been described in patients with hemophilia A until now. Authors represent a clinical case report of the development of the late-onset Vitamin K deficiency bleeding by a massive ICH in a patient with hemophilia A.