Sclerosing Epithelioid Fibrosarcoma (SEF) is a rare and distinct variant of low grade fibrosarcoma, found mainly in deep soft tissue of adult extremities. We report a case of Sclerosing Epithelioid Fibrosarcoma of soft tissue, which developed in a 69-year-old woman who presented a tumor involving the fourth finger of her right foot and which has not progressed much in size during the three months prior the surgical excision. Our patient is the hundredth case of Sclerosing Epithelioid Fibrosarcoma reported in literature since 1995, thus confirming the rarity of the tumor. Our experience showed that it is important to make an early diagnosis, in consideration of the clinical aggressiveness of this cancer. Another important aspect concerns the postoperative follow-up. The monitoring of PET-CT technique , although not standardized, could become part of proceedings of therapy and follow-up of tumor, thus allowing oncological radicality and avoid large amputations. To date, 24 months after diagnosis of SEF, our patient feels well, attends our outpatient clinic regularly and shows no evidence of relapse and/or metastasis