The authors report a case of neurothekeoma in a 16-year-old woman. The tumor was located in the dermis of the chin and exhibited a plexiform and multinodular architecture with dense lobules admixed with myxoid areas. Tumor cells were pleomorphic and few atypias and mitoses were seen. On immunohistochemical study, intracytoplasmic staining was observed with anti-vimentin antibody, and, to a lesser extent, with KP1 antibody. Few elements expressed S100 protein. Ultrastructural analysis showed undifferentiated mesenchymal cells. A review of 156 cases (of which 123 cases are reported in two main series) shows that neurothekeoma preferentially affects dermis in cervicofacial areas and shoulders in young women. It has a benign course and recurrence is not seen provided excision is complete. Two main histological varieties are described, i.e. cellular and myxoid, the latter being referred to as nerve sheath myxoma. Diagnostic problems can be raised with other nerve sheath tumors and melanocytic and fibrohistiocytic proliferations. Transition forms between neurothekeoma and neurinoma or neurofibroma are described. Despite confusing immunohistochemical and ultrastructural data, most authors admit that neurothekeoma can express schwannian or perineurial differentiations, and may also be derive from an undifferentiated mesenchymal cell of neural crest origin.