Neurothekeomas are benign tumors probably of nerve sheath origin and are also known as dermal nerve sheath myxomas. They are commonly found on the face, arm, or shoulder and less frequently the lower limbs. To our knowledge, this is the first case of a subungual neurothekeoma affecting the big toe. Histology confirmed a well-circumscribed, multilobulated tumor composed of bland stellate and spindle cells in a copious myxoid matrix staining positive with S100 protein.