Background: Basal cell carcinoma (BCC) in the nipple area is extremely rare and can be mistaken for Paget’s disease. Case Report: A 47-year-old woman complained of a slowly growing, scaly erythematous plaque around her right nipple which had developed over several years. Paget’s disease of the breast was suspected. Biopsies revealed a superficial BCC. The maximum tumor thickness was measured as 1 mm. Considering the superficial character of the tumor, photodynamic therapy (PDT) as a non-invasive approach was performed. Complete remission (CR) with an excellent cosmetic result was achieved after 4 PDT sessions. In the follow-up, CR with no signs of a local recurrence was evident after more than 24 months. Conclusion: In similar superficial cases of BCCs, non-invasive procedures such as PDT can be discussed as an alternative option to radical surgery or radiotherapy, with an excellent aesthetic outcome in carefully selected cases.