Primary eccrine adenocarcinoma is a rare malignant cutaneous adnexal tumor most commonly arising in the head and neck. Eccrine adenocarcinoma does not have diagnostic clinical features, and histologic recognition becomes important to consolidate the diagnosis of this tumor. Due to the small number of reported cases in the literature, optimal treatment modality has yet to be established. Earlier, surgical resection with wide local excision was performed for most of the cases. Mohs micrographic surgery (MMS), which becomes popular for treating infiltrative skin cancers, can be suggested as another treatment option for this rare malignant tumor of the skin. We have experienced five patients (M = 4; F = 1) with primary eccrine adenocarcinoma of the skin. Four patients had facial lesions and one had the tumor on the interdigital finger web space. Mean patient age was 50.4 ± 14.6 years (range, 29-70 years). Whole-body positron emission tomography revealed no other visceral neoplasm. Sentinel lymph node biopsy or lymph node dissection was performed if clinically indicated. All five patients were treated by MMS using frozen sections. Local recurrence occurred in one patient who were referred to our clinic and underwent MMS after initial recurrence. The remaining four patients showed no recurrence and metastases of the tumor during the follow-up period.