We report on a rare case of angiolymphoid hyperplasia with eosinophilia (ALHE) arising from the facial artery, which was pre-operatively diagnosed as an aneurysm. ALHE is a rare lesion, the precise nature of which remains controversial: it is still unclear whether ALHE is a tumour or an inflammatory disease or whether it can be separated from Kimura’s disease based on its pathological characteristics. ALHE rarely occurs in muscular arteries, and this is the first description of ALHE arising from the facial artery. Angiography vividly revealed the pathological structure of the lesion in this case, which prompted us to advocate angiography for diagnosing ALHE arising from muscular arteries. Complete dissection and cryotherapy proved to be effective treatment, although complete management of any therapeutic protocol should avoid recurrence of the lesion, whatever specific treatments are selected.