Haemophilia A (factor VIII [FVIII] deficiency) is a rare X-linked hereditary bleeding disorder. Before the introduction of prophylaxis in the 1960s and safer FVIII concentrates during the 1980s, life expectancy was reduced due severe bleedings or viral infection (e.g., human immunodeficiency virus). Currently, people with haemophilia are getting older, and curiously cardiovascular disease is a common comorbidity among them. We describe a 49-year-old man with severe haemophilia A (FVIII less than 1%) who had lipothymia due to complete atrioventricular block in October/2015. He was a smoker. He had gout (allopurinol) and arterial hypertension (indapamide, losartan and clonidine). He did not have human immunodeficiency virus infection. Hepatitis C virus was treated with daclatasvir with sofosbuvir. Medtronic ADAPTA®S ADDRS1 pacemaker was implanted few days later, under supervision of a Haematologist. He received plasma-derived (pd) FVIII 30 IU/kg q12h for 3 days, starting before the procedure. There was no abnormal bleeding during the procedure or in the post-procedure period. A small haematoma at the puncture and the battery pack sites resolved in one week. There were no further complications. The patient became asymptomatic, with no report of bradycardia episodes. pdFVIII prophylaxis (20 IU/kg twice weekly) was maintained until currently, without bleeding or thrombotic events. Till now, only few cases of pacemaker insertion in people with haemophilia have been described. It is a minor procedure and a multidisciplinary approach can ensure its success with minimal bleeding risk.