An extraabdominal desmoid tumor of the shoulder occurring in a middle-
aged woman without Gardner's syndrome is described. Two punch biopsy s
pecimens from the tumor were initially interpreted as representing sca
r tissue; a third incisional biopsy specimen demonstrated the characte
ristic features of a desmoid tumor. Because desmoid tumors are locally
aggressive, early diagnosis and treatment are crucial to minimize mor
bidity and mortality. Typical clinical and histologic findings charact
eristic of an extraabdominal desmoid tumor are described, and treatmen
t options are reviewed.