Apocrine gland carcinoma is a rare form of sweat gland neoplasm with a distinctive cytologic appearance. Although the region of the axilla remains the most common site for these tumors, apocrine gland carcinoma of the anogenital region, eyelid, ear, chest, wrist, lip, foot, toe, and finger have been reported. Classically, these slow-growing lesions present as painless, colorless or reddish, firm or cystic nodules. More than half of the reported patients with apocrine carcinoma had lymph node metastases at the time of diagnosis. Wide local excision is standard therapy for these lesions. A therapeutic lymph node dissection is indicated for confirmed lymph node metastases and may have a role in the setting of a large or highly aggressive tumor with narrow surgical margins. Although apocrine gland carcinoma responds poorly to chemotherapy, adjuvant radiotherapy may be used in advanced local or regional disease. The authors describe a 69-year-old man with a large recurrent apocrine gland carcinoma of the axilla treated with en bloc excision with axillary dissection and offer a pertinent review of the English literature and recommendations for treatment.