By analyzing the clinical data of patients with cerebral amyloid angiopathy related inflammation (CAA-ri), the heterogeneity in clinical and imaging features, prognosis or other aspects was summarized, so as to guide the diagnosis and treatment of the disease. Methods: Clinical data of 6 CAA-ri inpatients or outpatients in the Department of Neurology in our hospital from 2019 to 2021 were enrolled. All the patients underwent cranial MRI scans, of which 3 underwent cerebrospinal fluid related examination, 2 underwent APOE genotype detection, and 2 underwent pathological biopsy. Results: The clinical heterogeneity includes the diversity of disease course, symptoms and signs. CAA-ri may be acute, subacute or chronic disease, or a rare course of attack. Symptoms such as cognitive impairment, limb weakness, speech impairment and mental behavior abnormality are frequently seen. There were common features of asymmetrical white matter lesions, microhemorrhage and hemosiderin deposition in MRI, but the range and degree of lesions varied greatly. Hormone and other immune therapies are effective in severe cases. Mild patients may have a self-limited course, but are prone to recurrence. Conclusion: CAA-ri has a high degree of clinical heterogeneity, but the common features of its imaging findings can highly suggest diagnosis, and hormone therapy can effectively assist further clinical diagnosis.