Aneurysmal fibrous histiocytoma is often clinically misdiagnosed. In this study, we put forward an insight on how to help diagnose this disease clinically. A retrospective chart review was performed on all patients diagnosed with aneurysmal fibrous histiocytoma from 2007 to 2017 in the Department of Dermatology, Union Hospital,
China, and all clinical data were collected from the hospital archives. From a total of 418 patients diagnosed with cutaneous fibrous histiocytoma, only 30 patients were confirmed to have aneurysmal fibrous histiocytoma out of which only 2 patients were clinically diagnosed with aneurysmal fibrous histiocytoma. The remaining 28 patients were diagnosed with various types of vascular tumors although pathology classified them as having aneurysmal fibrous histiocytoma. Among the 30 patients, 9 were male and 21 were female. There were following age groups: 13-19 (mean 16, n=4), 20-29 (mean 26.25, n=8), 30-39 (mean 33, n=7), 40-49 (mean 44, n=4), 50-59 (mean 56.75, n=4), 60 and above (mean 61, n=3). Tumors were present on the head, neck, back, waist, hips and upper and lower extremities. After complete excision, there was no recurrence and no complications. Histologically, lesions showed the typical pseudoangiomatoid spaces without endothelial lining and infiltration of fibrohistiocytes in hemosiderotic pigmentation. It was suggested that although the prognosis of aneurysmal fibrous histiocytoma is good, accurate diagnosis is paramount to avoid clinical misdiagnosis and subsequent complications.