This report describes a new entity which has been defined as infundibular carcinoma (IC) of the skin. This new entity must be distinguished from other basaloid carcinomas arising from the epidermis, the hair follicles or from sweat duct and glandular elements. Specific criteria have been used to define ICs. These include the macroscopic and the microscopic site of the tumour as well as its histomorphological and immunohistochemical patterns. A brief review of benign and malignant tumours of the pilar unit and the differential diagnosis of IC is also provided.