Basal cell carcinoma (BCC) is the commonest malignant neoplasm in humans. Although a histopathological diagnosis of BCC is straightforward in the vast majority of cases, unusual histological variants can present a diagnostic challenge. A small proportion of BCCs show features which are generally associated with cutaneous adnexal neoplasms. Such changes may involve either the epithelium or the stroma and can mislead the pathologist particularly in small biopsies. Despite the growing evidence which speculate that BCC is a primitive follicular tumor, it is unusual to encounter tumors which actually show definitive signs of adnexal differentiation. This review aims to address this somewhat overlooked aspect of a very common tumor and offers practical guidance to distinguish them from adnexal neoplasms which they might mimic.