Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a life-threatening syndrome of aggressive thrombosis, often profound thrombocytopenia, and frequently overt disseminated intravascular coagulation. It has been associated with 2 adenovirus vector COVID-19 vaccines: ChAdOx1 nCoV-19 (AstraZeneca) and Ad26.COV2.S (Janssen). Unlike the myriad of other conditions that cause thrombosis and thrombocytopenia, VITT has an important distinguishing feature: affected individuals have platelet activating anti-PF4 antibodies that appear in a predictable time frame following vaccination. The reported incidence of VITT differs between jurisdictions; it is dependent on accurate ascertainment of cases and accurate estimates of the size of the vaccinated population. The incidence ranges from 1 case per 26,500 to 127,3000 first doses of ChAdOx1 nCoV-19 administered. It is estimated at 1 case per 518,181 second doses of ChAdOx1 nCoV-19 administered, and 1 case per 263,000 Ad26.COV2.S doses administered. There are no clear risk factors for VITT, including sex, age, or comorbidities. VITT is a rare event, but its considerable morbidity and mortality merit ongoing pharmacovigilance, and accurate case ascertainment.