| Summary: | Introduction and importance: Dermatofibrosarcoma Protuberans (DFSP) is a rare and fatal variant of Spindle Cell Sarcoma. It has an annual incidence rate of 0.8 to 4.5 cases per one million individuals. It's locally aggressive and has vague and masquerading clinical presentations. Misdiagnosis is devastating as it can lead to time wasting, expenditure of unnecessary resources, and possibly raise morbidity and mortality for patients. It is warranted to raise preoperative clinical awareness to achieve prompt surgical therapeutic interventions to reach an up-to-par prognosis. Case presentation: We demonstrate the case of a 50-year-old previously healthy Middle Eastern male patient, who was referred to our General Surgery clinic with the chief complaint of an expansive bulge in his left iliac fossa. Preoperative imaging could not exclude a neoplastic cause behind the presentation. Based on the clinical picture, a surgical intervention was decided. Clinical discussion: Our patient's treatment was consummated by means of classical surgical resection of the lesion with adequate negative margins and referring him to an oncologist specialized in DFSP to undergo the necessary adjuvant treatment. Definitive diagnosis was firmly entrenched postoperatively after finalization of the histopathological and immunohistochemical analyses of the resected protuberance. Conclusion: DFSP is an eminently rare entity, especially DFSPs which originate from a surgical scar -as was our patient's- and fluctuates in its clinical presentation, thus, it is our responsibility to depict, study this malignant tumor, and document its incidence, so that we can make ironclad clinical decrees to plummet the morbidity and mortality of this relentless neoplasia. © 2022 The Authors
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