We present a case of angiolymphoid hyperplasia with eosinophilia (ALHE) in the occipital area in a 30-year-old Japanese woman. Histological examination revealed vascular proliferation with cellular infiltration composed of lymphocytes, eosinophils, and mast cells. Strong pulsation at the occipital area was apparent and a 3-dimensional computed tomographic scan demonstrated anomalous dilatation of the left occipital artery. The skin lesion partially responded to the embolization of the left occipital artery.