Acantholytic anaplastic Paget disease is a subset of classic Paget disease with distinctive histologic features that may be a diagnostic challenge. We report a case of this rare variant of Paget disease and briefly review its characteristics. An 81-year-old woman presented with an asymptomatic lesion on her left nipple of 2 years’ duration. Physical examination revealed enlargement of the left nipple with an erythematous, eroded, exudative, and lightly crusted surface. The areola was unaffected. On physical examination a poorly defined, tough but lightly mobile mass was observed on the upper and inner quadrant of the ipsilateral breast.