BACKGROUND: Thrombotic microangiopathy (TMA) is a rare disease, and kidney pathology is diagnosis gold standard. Researches regarding long-term outcomes of malignant hypertension (MHT)-related TMA are sparse. METHODS: The retrospective cohort was conducted in a single medical center in southern Taiwan. Individuals with kidney pathology proven TMA between January 2000 and December 2019 were enrolled. Etiologies of TMA were categorized into MHT and others. Baseline demographics and clinical parameters were compared between individuals with MHT-related TMA and other TMA. Statistical analyses were performed in September 2022. Kaplan-Meier survival analysis was used to demonstrated survival, end stage renal disease (ESRD)-free rate, and ischemic stroke events of individuals with TMA. RESULTS: Of 23 participants, with mean follow-up period of 3.9 years, 5 individuals (21.7%) had MHT-related TMA (5 men [100.0%]; 43.2 ± 4.9 years old). The other 18 individuals had other causes-related TMA (three men [16.7%]; 48.7 ± 17.0 years old). The baseline characteristics between groups were balanced except that MHT-TMA group had higher proportion of male (5 individuals [100%] vs. 3 [16.7%], P = 0.001), lower proportion of fragmented red blood cell (0 individuals vs. 8 [61.5%], P = 0.03), and higher serum creatinine (9.4 mg/dL vs. 4.1 mg/dL, P = 0.001). Individuals with TMA had a mean survival of 11.39 years and mean ESRD-free period of 4.15 years, while no individuals experienced ischemic stroke. CONCLUSION: Kidney pathology proven TMA comprised various etiologies, and MHT is the most common etiology. Large scale study is needed to better understand the association of different etiologies and clinical outcomes.