CLINICAL CASE: We present the case of a 63-year-old female patient with a history of sensation of mass and increased volume in the abdomen (hypogastrium). She underwent a tomography scan showing an 11 cm mass in the hypogastrium with an oval shape of soft tissue density with heterogeneous reinforcement and well-defined contours. The patient was taken to the operating room where she underwent exploratory laparotomy, resection of the mesenteric tumor, and resection of the small intestine segment with anastomosis. Subsequently, histological confirmation of desmoid tumor is given from the pathology sample. The patient progressed satisfactorily and was discharged on the 5th postoperative day.

REASON FOR REPORT: Desmoid tumors are extremely rare and usually non-life-threatening neoplasms, but they have a locally aggressive behavior and variable clinical course that can affect the patient’s functional capacity and quality of life. The clinician's suspicion in the presence of abdominal masses and antecedents, the differential diagnosis, imaging studies, histopathology and immunohistochemistry play a key role in the diagnosis. Treatment must be multidisciplinary and individualized depending on each patient and guarantee adequate follow-up due to the high recurrence rate of these tumors.

CONCLUSION: Desmoid tumors are characterized by locally aggressive behavior, infiltrative growth, high local recurrence rate due to the lack of capsule, and metastasis absence. They are infrequent benign pathologies so; their diagnosis and treatment are a medical challenge.