BACKGROUND: Fibroepithelioma of Pinkus (FeP) is an uncommon and controversial skin lesion, sharing features of both basal cell carcinoma (BCC) and trichoepithelioma. In this article, we present a case of FeP and synthesise current concepts on the etiopathogenesis, diagnosis and treatment of this uncommon tumour.CASE REPORT: We report the case of an 88-year-old male patient presenting to the dermatology clinic for a sharply demarcated, pink, exophytic cutaneous tumour situated in the left inguinal region. The histopathological examination performed after complete surgical excision of the lesion revealed a diagnosis of FeP. A systematic review of the literature was conducted. The terms `fibroepithelioma` and `Pinkus` have been searched in bibliographical databases, including PubMed and Google Scholar, without time limitation up to February 15th, 2017. Seventy-nine articles that fulfilled all the required conditions were identified. Relevant citations and additional articles identified from references have been assessed. The systematic review included a total number of 452 cases of FeP.CONCLUSION: Even though FeP is considered a relatively rare tumour, its true incidence rate might be higher than previously believed. The clinical aspects of the lesion described in this paper and its location in the left lower quadrant of the abdomen are classic features of FeP. Histopathologic examination revealed features of both BCC and trichoepithelioma. Further epidemiological studies are required to clarify whether patients with FEP should be screened for the occurrence of other malignancies.