Abstract Introduction Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade sarcoma affecting dermis and subcutaneous fat. Typically presents as slow-growing lump on the trunk, extremities or head and neck.(1) It can be locally aggressive with a high recurrence rate.(2) Only 6 reported cases of DFSP in peri-anal region. Case Description A 26-year-old male presented with slow-growing peri-anal lump for over a year with intermittent localised pain and episodes of bleeding. No other symptoms or trauma to peri-anal area noted. No relevant medical or surgical history. Examination revealed an irreducible two-by-five by one-centimetre semi-pedunculated perianal lump extending from the seven o’clock to eleven o’clock position. It was firm and tethered to the skin. Case Description Flexible sigmoidoscopy was normal. Excision biopsy histology reported diffuse infiltration of spindle cells with storiform pattern of growth without clear margins. Immunohistochemistry analysis expressed CD34 with minimal focal expression of SMA and variable Ki-67 proliferation indexed up to 15-20% in most active areas. SOX10, S100, desmin, factor XIIIA, MNF116 and CD31 were negative. Diagnosis was DFSP. Local lower gastrointestinal multi-disciplinary arranged urgent imaging and referral to tertiary centre. Magnetic resonance imaging of the pelvis identified no definite underlying localised restricted diffusion or solid enhancing nodule. Wide local excision at tertiary centre with histopathology confirmed clear margins. Conclusion Patient is under follow-up locally and at the tertiary. Urgency and a singular episode of stool incontinence was reported at two-weeks follow-up which subsequently normalised. He is currently under 3-6 monthly reviews with repeat MRI prior to next appointment. Patient has returned to normal activities.