Desmoid-type fibromatosis is categorized as a locally aggressive but non-metastasized tumor in WHO classification. This tumor is deep-seated myofibroblastic neoplasm and characterized by infiltrative growth pattern which is resulted in high local recurrence after surgical removal (Fletcher et al., WHO classification of tumours editorial board. Soft Tissue and Bone Tumours. IARC Press, Lyon, 2020). Recent guideline proposes the treatment algorism composed of active surveillance and active treatment including surgical removal and chemotherapy depends on the anatomical site, progression, and symptom (Desmoid Tumor Working Group, Eur J Cancer 127:96–107, 2020; Smith et al., Ann Surg Oncol 25(6): 1544–1554, 2018). Here we present two cases of desmoid-type fibromatosis, which was managed by an active surveillance (Case. 1) and low-dose methotrexate plus vinblastine chemotherapy (Case 2).