Three cases of dermal nerve sheath myxoma have been studied by light microscopy, histochemistry and immunohistochemistry. The pertinent literature has been reviewed. This lesion, which is a rare benign tumour of probable nerve sheath origin, arises most often in young adults and shows a predeliction for females. Histologically it has a characteristic appearance. Histochemically, the heteroglycan content of the mucoid matrix is more in keeping with a cartilaginous lesion. The histogenesis and differential diagnosis are discussed.