Angiolymphoid hyperplasia with eosinophilia (ALHE), also named epithelioid hemangioma (EH), is an inflamed vascular tumefaction of uncertain pathogenesis, characterized by proliferation of histiocytoid endothelial cells with prominent lymphocytic and eosinophilic infiltration. Although considered a benign condition, it may recur in up to one-third of cases in the absence of complete surgical excision. The pathogenesis of ALHE/EH is still controversial. However, reaction to trauma and arteriovenous shunting are considered relevant. Histologically, ALHE/EH may be differentiated from other vascular neoplasms by its several unique characteristics including prominent proliferation of plump endothelial cells, and accompanying eosinophilic and lymphocytic inflammation, often with formation of lymphoid follicles. Surgery is the mainstay of treatment and various other treatment strategies have been used with varying results.