Paget’s disease of breast is a cutaneous malignancy of the breast involving the nipple-areolar complex that is often associated with underlying neoplastic lesions of breast parenchyma. This condition is often mistaken for a wide range of dermatological conditions, leading to delay in diagnosis. This review article revisits the etiology, clinical presentation, differential diagnosis, diagnostic work-up, natural history, management and prognosis of Paget’s disease of breast.