Background: Epitheloid sarcoma has been described in 1970 by Enzinger
as a separate tumor entity. It is a rare soft-tissue tumor mostly foun
d in the distal extremities in young adults. The head and neck region
is only very infrequently affected. Patient and result: We report the
rare case of a manifestation of an epitheloid sarcoma in the area of t
he right parotid gland in a 72-year-old patient; Only two more cases w
ith a similar tumor entity and location have been described in the int
ernational literature. The patient underwent total parotidectomy, radi
cal neck dissection, and postoperative radiotherapy two years ago and
has been free of recurrence since then. Conclusions. Epitheloid sarcom
as of the head and neck are very rare tumors. They are treated by radi
cal local tumor removal, neck dissection, and postoperative radiothera
py. Prolonged postoperative follow-up is necessary since recurrences c
an occur after up to more than 15 years.