Neurothekeoma is a rere cutaneous tumor that was first described by Gallager and Helwig⁵⁾ in 1980. On histological finding, this tumor is most closely related to the neurofibroma, although it is not certain whether their cells resemble the perineural cell or the classic Schwann cell, but the positive staining for S100 protein suggests that neurothekeoma is a tumor of Schwann cell origin. We present a typical case of neurothekeoma arising on the retroauricular skin, an unusual site for this tumor, in a 19-year-old man.