Extra-abdominal desmoid tumors (EADTs) are rare soft tissue tumors that mainly affect young adult females. The origin of these tumors is multifactorial. Clinical and radiological signs are non-specific. Diagnosis is based on histological examination and immunohistochemical studies. In cases where diagnosis is difficult, molecular biology is recommended. The evolution of these tumors is marked by the occurrence of local recurrence. The occurrence of a second distant localization is exceptional. Due to the rarity of these tumors, treatment strategies have not yet been standardized, making it difficult to conduct large randomized trials. The aim of this paper is to clarify the clinical and radiological aspects of TDEA, to study the histological and immunohistochemical characteristics of these tumors and to discuss their therapeutic and evolutionary modalities.