Abstract : Desmoid tumor is a rare disease that originates from the proliferation of fibrous tissue. Intra-abdominal desmoid tumors are sporadic or associated with specific familial syndromes, such as Familial Adenomatous Polyposis Syndrome (FAP). These tumors are often challenging to diagnose, and active surveillance is also essential given the high recurrence rate despite adequate treatment. We present the case of an 18-year-old female with a recurrent intra-abdominal desmoid fibromatosis tumor despite surgical resection combined with radiotherapy. The recurrences are treated with anthracycline chemotherapeutic regimens. There is a diagnostic delayed due to nonspecific presentations. Immunohistochemistry assay is a cornerstone for accurate histopathological diagnosis, which guides treatment. The mainstay of treatment is surgical resection; chemotherapy and radiotherapy have a role in managing both primary and recurrent lesions.