Desmoid tumors (DTs) are rare soft tissue neoplasms, especially when originating from the small bowel. An association with familial adenomatous polyposis (FAP) of the colon has been well documented. Within the FAP population, there is a strong correlation between prophylactic procto-colectomy and subsequent development of DTs. Very few reports describe cases of FAP initially presenting with desmoid-related complications. Therefore, we underwent a review of the literature in order to delineate the characteristics of desmoid tumors occurring as an initial presentation of FAP.