Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare disease, currently considered a benign vascular proliferation of unknown etiology, the treatment of which is still unclear and challenging. It affects mostly women in the age range from 20 to 40. The lesions may be asymptomatic, itchy, painful, haemorrhagic and pruriginous. Although the etiology of ALHE remains unknown, trauma is considered to be the most likely cause. Angiolymphoid hyperplasia with eosinophilia can be diagnosed by a correlation between patient's history, clinical and radiographic findings supported by histopathological examination. There is a wide spectrum of treatment such as intralesional therapy (sclerosant and steroid), and surgical and non-surgical treatment modalities. Spontaneouos remission and local recurrence can be monitored. Hereby we presented a rare case of ALHE on retroauricular region treated by intralesional therapy and reviewed the relevant literature.