Desmoid tumors (DTs) of the head and neck have typically been classified as extra-abdominal, although the anatomic challenges of the head and neck warrant consideration of these DTs as a special entity. We present a review of DTs and describe our series of five patients with DTs of the head and neck treated within 2 decades. Altogether, 53 patients with DTs treated surgically at a tertiary care center over a 20-year period were retrospectively reviewed. Outcomes of the treatment of DTs of the head and neck (n = 5) were analyzed as a case series. DTs are rare, histologically benign, but locally aggressive tumors. In our series of 5 patients with head and neck DTs, no patient experienced a recurrence during a median follow-up of 47.5 months (range 13 to 150), although all had positive histologic margins. The functional integrity of vital structures over meticulous radicality of the tumor resection must be considered, especially in the head and neck.