Background: Pyoderma gangrenosum is a rare neutrophilic dermatosis which leads to necrotic and painful skin ulceration. PG of the breast is extremely rare with 32 documented cases in the current literature. Delay in diagnosis worsens scarring as the ulcers are rapidly expanding, painful and usually slow to heal. Case presentation: We present a case of pyoderma gangrenosum of the breast in a patient with associated rheumatoid arthritis which was initially diagnosed as an infected breast ulcer and later successfully treated with systemic steroids and intravenous immunoglobulin (IVIG). Conclusion: Even though PG of the breast has been gaining increased recognition over the past two decades, this has been more common in the post-surgical setting. This case highlights the need to consider PG as a differential diagnosis when faced with unsual cases of breast ulceration and the importance of multidisplinary approach for effective treatment of this condition.