Basal cell carcinoma of the nipple areola complex in female patients is an extremely rare tumor with a malignant clinical behavior. The Authors present the case of a Basal cell carcinoma of the nipple areola complex in a Caucasian woman, evaluated by clinical and histological findings. Our clinical experience suggests that Basal cell carcinoma of the nipple areola complex has a local aggressive behavior with an higher metastatic potentiality compared to basal cell carcinoma of common sites. However, an accurate tissue sparing excision may be successful in the long term.