Pyoderma gangrenosum is a cutaneous ulcer diagnosed by exclusion. The underlining causes of pyoderma gangrenosum are numerous. Malignancy is a well-known underlying cause. Nonetheless, breast cancer is an uncommon cause, with few reported cases in the literature. There is no specific treatment of paraneoplastic pyoderma gangrenosum, apart from treating the underlying malignancy. Herein, we report a case of pyoderma gangrenosum with underlying breast cancer, successfully treated by systemic steroids, although the patient showed no response to anticancer therapy and expired shortly after the resolution of the cutaneous lesion. Further studies are needed to conclude the benefit of systemic steroids in paraneoplastic pyoderma gangrenosum, yet we recommend a trial of systemic steroids in the case of generalized resistant paraneoplastic pyoderma gangrenosum.