Congenital thrombotic thrombocytopenic purpura (cTTP) is an ultra-rare disorder caused by severe ADAMTS13 deficiency [1]. Between 2001 and 2020, only 226 patients with cTTP were reported worldwide [2]. As of 2021, we identified 67 patients with cTTP in Japan alone [3,4]. Theoretically, patients with cTTP are at a high risk for thrombosis due to COVID-19 infection. This is attributed to the increase in plasma von Willebrand factor levels and decrease in plasma ADAMTS13 activity with disease severity in patients with COVID-19 infection [5].Recently, Dykes et al. reported a case in which COVID-19 vaccination exacerbated the cTTP condition [6]. This patient developed an acute ischemic stroke with mild thrombocytopenia shortly after receiving the COVID-19 vaccine (mRNA-1273). To date, over 10 cases of de novo immune TTP have been reported following COVID-19 vaccination worldwide [7,8]. However, there have been no other reports of COVID-19 vaccine-induced flare of cTTP. In Japan, two types of mRNA-based vaccines, BNT162b2 (Pfizer-BioNTech) and mRNA-1273 (Moderna), are available. We administered a questionnaire survey in Japanese patients with cTTP regarding the adverse events after vaccination in October 2021, when double doses of vaccinations were almost completed in Japanese applicants who were ≥ 12 years of age.The questionnaire was sent to physicians who were treating patients with cTTP. Of the 67 Japanese patients with cTTP, 58 were alive and 6 were under 12 years of age. According to the questionnaire survey of 42 patients, 38 had completed vaccination twice, and 4 patients had not * Masanori Matsumoto