Familial gigantiform cementoma is a rare autosomal dominant tumor that is b
enign but can result in disfigurement of the facial skeleton. Two families
with a total of five patients presented for treatment. Because of a lack of
opportunity to obtain treatment early, three of the patients presented in
adult life with massive tumors requiring extensive resection and complex re
construction in multiple stages. The two female patients had chronic anemia
caused by multifocal polypoid adenomas of the uterus and required hysterec
tomy before treatment The last three patients had elevated alkaline phospha
tase levels before turner resection, and these levels decreased after surge
ry. With extensive resection of the tumors and reconstruction of both the s
oft tissues and facial skeleton, goad functional and aesthetic results can
be obtained. There has been no tumor recurrence with 3 years of follow-up.