Aggressive digital papillary adenocarcinoma is a rare neoplasm of eccrine sweat gland origin that typically presents as a mass on a finger, toe, or the adjacent skin. Less than 100 cases have been reported. The majority of these cases are described in males in their fifth to seventh decade. We report a case of an aggressive digital papillary adenocarcinoma of the right second toe in a 15-year-old white female. A metastatic work-up, computed tomography of the chest, abdomen, pelvis, and a bone scan, was negative. The patient underwent amputation of the right second toe through the metatarsophalangeal joint. Two sentinel lymph nodes were biopsied and found to be negative for metastatic disease. One year after surgery the patient has no evidence of disease recurrence. To our knowledge, this is the youngest reported case of an aggressive digital papillary adenocarcinoma.