Introduction Porocarcinoma is an aggressive cutaneous carcinoma arising from the intraepidermal component of the sweat glands. Given its uncommon nature, only a limited number of studies have addressed this issue. This study summarizes the different presentations and management of the disease. Methods The eligible databases were searched to identify English-language studies published up to January 16th, 2024. The inclusion criteria comprised studies that focused on the presentation and management of eccrine porocarcinoma, with a clear statement of the outcomes of the chosen management. The data collected from the studies included the first author's name, country of study, type of study design, patient demography, the clinical presentation of the tumor and its location, histopathological findings, metastasis status, treatment strategy, and the subsequent prognosis. Results The initial search yielded 817 papers, with only 22 meeting the inclusion criteria. The studies comprised 1004 patients with a mean age of 78.7 years. In total, 527 (52.5%) of them were male and 461(45.9%) were female. The most commonly affected regions were the head and neck followed by the lower extremities. The most frequent treatment option was surgical excision in 876 (87.3%) patients. Distant metastasis accounted for about 2%, while lymph node involvement, occurred in 36 cases (3.6%). A good prognosis was reported in 57.3% of the cases. Both the age and tumor size had a significant effect on prognosis (p <0.05). ConclusionThe lesion can develop anywhere on the body, with a survival rate exceeding 50%. Wide-based surgical excision remains the predominant treatment option.