Abstract Background Desmoid-type fibromatosis (DF) is a rare disease, which often occurs in young adults. Medical treatment is an important option in the treatment algorithm of DF. Different chemotherapeutic regimens showed clinical activity in DF, but overall treatment tolerability remains poor for this patient cohort. Novel approaches investigated tyrosine kinase inhibitors in DF, but tolerability remained an issue. Case presentation We treated a patient with progressive DF after failure of chemotherapy for 1 year with singe agent bevacizumab. He achieved a symptomatic and radiologic response while attainning excellent tolerability. Conclusions This is the first report on single agent bevacizumab in DF, which showed both, good tolerability and efficacy in our patient, thereby warranting future trials in DF.