Marjolin’s ulcer is rare and aggressive malignancy. The objective was to conduct a review of Marjolin’s ulcer cases to gain a better understanding and its management. A retrospective study of patients with Marjolin’s ulcers at our hospital between 2010 and 2019 was conducted. For the systematic review, the electronic database of the National Library of Medicine was searched and articles published between 2000 and 2019 were included. A total of fourteen patients were included in the retrospective study. Twelve patients were male. The mean age was 59.71 years. The latency period was 27.78 years. The most common malignancy was squamous cell carcinoma, and 71.43% had developed on the lower extremities. Well-differentiated squamous cell carcinomas occurred in 38.46% of the cases Rates of lymphovascular and perineural invasion were 30.77% and 23.08%, respectively. Results from the literature search yielded 80 case reports (91 cases) and 42 retrospective reviews (1216 cases). Most cases were male and the most common location was lower extremities. The latency period for the acute and chronic periods was 2.75 months and 20.75 years, respectively. The most common malignancy was squamous cell carcinoma (92.27% in the retrospective reviews) and 59.98% of the cases exhibited well-differentiation. Rates of regional lymph node involvement and distant metastases were 10.20% and 12.09%, respectively. Marjolin’s ulcer in 21 st Century is not so rare. The most common is well-differentiated squamous cell carcinoma. Early flap surgery or skin grafts for chronic ulcers or burn scars are recommended to reduce risk. Guideline for surgical management is established.