BACKGROUND The optimal management of inoperable desmoid tumors is still unclear. We report a 26 year‐old female patient with familial adenomatous polyposis suffering from a recurrent inoperable intraabdominal desmoid tumor and its sequential treatment. METHODS Treatment strategies included low‐dose tamoxifen (30 mg orally per day), high‐dose tamoxifen (90 mg orally per day), and a subsequent combination of goserelin acetate (3.6 mg subcutaneously once every four weeks) plus low‐dose tamoxifen, medroxyprogesterone acetate (1000 mg orally per day) and interferon gamma (3 Mio IU subcutaneously 3 times a week). RESULTS The combination of goserelin acetate and low‐dose tamoxifen resulted in a decrease in tumor size and a complete relief of symptoms for 17 months. Thereafter the tumor progressed and again growth was stopped with interferon gamma therapy for another 6 months. All other treatment modalities had no effect. CONCLUSIONS This study demonstrates long‐term regression of a desmoid tumor with combined endocrine therapy using goserelin acetate plus tamoxifen. Tumor progression after 17 months was again stopped by a combination of interferon‐gamma and goserelin acetate. Cancer 1996;77:1061‐5.