Basal cell carcinoma (BCC) is a slow-growing, locally invasive form of skin cancer originating from basal cells, and it accounts for 75% of non-melanoma skin cancers. BCCs exhibit various clinical presentations, including nodular, superficial, morpheaform, and others. Most of these tumors appear in sun-exposed areas of the face and neck, while their occurrence in the nipple-areola complex is rare. When BCC arises in these areas, Paget’s disease of the breast must be excluded as a possibility. Furthermore, the development of BCCs at a young age, especially with an unusual distribution or multiple lesions, should prompt consideration of genetic conditions such as basal cell nevus syndrome. This case report describes a rare instance of superficial BCC in the nipple-areola complex, initially mistaken for Paget’s disease, ultimately leading to the diagnosis of basal cell nevus syndrome.
