Background <p>Pleomorphic dermal sarcoma is a rare form of skin cancer affecting the cutaneous layer. It is associated with recurrence and metastasis, is seen in elderly patients, and is more common in the head and neck. The reported incidence rate is 0.152 cases/100,000. Differentiation of pleomorphic dermal sarcoma requires extensive immunohistochemistry, as its diagnosis depends mainly on exclusion, ruling out other malignancies. This paper reviews our current practice in the management of pleomorphic dermal sarcoma and the literature on this newly diagnosed entity of skin cancer.</p> Methods <p>This was a retrospective study of patients diagnosed with pleomorphic dermal sarcoma in our unit between 2016 and 2023. The primary lesion sites, surgical excision margins, investigations undertaken, recommended adjuvant therapy, follow-up plans, recurrence, and metastatic rates were reviewed.</p> Results <p>Data collected from 19 patients between 2016 and 2023 revealed that the disease predominantly affects men and lesions mainly on the head and neck. The diagnosis requires extensive immunohistochemistry to differentiate it from other malignancies. The recurrence rate was 21% and the need for adjuvant therapy was approximately 42.1% of the cases.</p> Conclusions <p>Pleomorphic dermal sarcoma (PDS) is a rare malignancy. The management guidelines are not yet fully clear concerning the investigations required, their frequency, role of adjuvant therapy, and frequency and duration of the necessary follow-up. Our practice and literature review may help ascertain the need for the establishment of national guidelines.</p> Level of Evidence: <p>Level IV, risk /prognostic study.</p>