BACKGROUND Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare entity that usually occurs as 0.5-3 cm, pink-to-brown nodules on the skin surface and subcutaneous tissues of the head and neck (Al-Muharraqi MA, Faqi MK, Uddin F, Ladak K, Darwish A. Angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) of the face: an unusual presentation. Int J Surg Case Rep 2011;2:258-60.; Suzuki H, Hatamochi A, Horie M, Suzuki T, Yamazaki S. A case of angiolymphoid hyperplasia with eosinophilia (ALHE) of the upper lip. J Dermatol 2005; 32:991-5.). METHODS A 29-year-old woman presented to our ENT (ear-nose-throat) clinic with multiple, small reddish to pinkish nodules of approximately 1 × 2 cm2 in dimension. A biopsy from the surface of the left external auditory canal skin, where she had severe itching, showed features consistent with Angiolymphoid hyperplasia with eosinophilia ALHE. CONCLUSIONS Whether angiolymphoid hyperplasia with an eosinophilia etiology is a neoplastic or atopic hypersensitivity reaction (unusual reactive process) is controversial. The definitive treatment is surgical excision and follow-up (Al-Muharraqi MA, Faqi MK, Uddin F, Ladak K, Darwish A Angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) of the face: An unusual presentation. Int J Surg Case Rep 2011;2:258-60.; Barnes L, editor. Surgical Pathology of the Head and Neck. 3rd edn. New York: Marcel Decker; 2001.). CONCLUSIONS Although ALHE of <3 cm has been rarely reported in the literature, it should be considered in the differential diagnosis of large subcutaneous tumors of the head and neck.