Sweat gland carcinoma is a rare malignant tumor of the skin adnexa that can occur in a variety of locations. It is a slow-growing tumor that metastasizes early and often. The rarity of this tumor has led to a relative lack of adequate evidence with regard to treating this entity. Recent advances in immunohistochemical staining have better defined this tumor and have aided in diagnosing its subtypes though histologic grade and presence of regional lymph nodes at the time of diagnosis. These are the best indicators for prognosis and management currently available. Indications for sentinel node biopsy are currently not well defined. Because of the poor prognosis of high-grade sweat gland carcinoma, clinicians should be aware of this entity and be prepared to treat aggressively. Management of sweat gland carcinoma is difficult because extensive study into both surgical and nonsurgical treatment has not been done. We present a case of sweat gland carcinoma of the lower extremity and a review of the clinicopathologic literature of these rare neoplasms, including diagnosis and surgical management.