Abstract
Cowden syndrome, a rare genetic disorder estimated to occur in 1 in 200,000 live births and inherited as an autosomal dominant mutation in PTEN gene, is part of the PTEN hamartoma tumor syndrome. These patients are at risk of breast cancer, as well as cancers of the digestive tract, thyroid, uterus and ovaries. Often identified by their dentist due to characteristic papillomatosis in the gingival mucosa, they have an estimated lifetime risk of up to 81% of developing breast cancer. This article describes a relatively uncommon procedure of bi-pedicle nipple-sparing mastectomy, a modified Letterman technique, used in the setting of immediate implant based reconstruction in a patient with Cowden syndrome.