Invasive papillary carcinoma of the breast and Paget's disease are both rare entities. We report the first known case of invasive papillary carcinoma associated with Paget's disease in a woman who presented clinically with a nipple mass. While invasive papillary carcinoma has been reported to have an indolent course without need for overtreatment, our patient had unusual unfavourable histological features. Literature review of this rare subtype was performed.
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