Objective Epithelial angiosarcoma is a rare disease. In order to increase the understanding of clinical imaging, pathology, and immunohistochemical manifestations of epithelial angiosarcoma, and to improve the positive diagnosis rate of epithelioid angiosarcoma. Method A retrospective report of a case of chronic epithelioid angiosarcoma of chronic sclerosing osteomyelitis from our department, considering the possibility of occupational exposure, and discussing its imaging and pathological manifestations. Result Bone tuberculosis and old fractures were considered and targeted treatment was not effective. After targeted treatment according to epithelial angiosarcoma according to pathological results, the patient recovered and was discharged. It is not ruled out that exposure to hair dyes is one of the causes, and it is worth following up. Conclusion Epithelial hemangiosarcoma secondary to chronic sclerosing osteomyelitis is rare, with a high misdiagnosis rate of imaging examination, a gold standard for pathological diagnosis, and immunohistochemical detection is of auxiliary diagnostic significance.