Digital papillary adenocarcinoma (DPA) is a rare malignant eccrine tumor. A 62-year-old female presented with a subcutaneous nodular 1.5cm-mass in the thumb. Macroscopically, a poorly circumscribed mass containing cystic and solid components was observed. Microscopically, epithelial neoplasm consisting of tubular-cystic structures with back-to-back arrangements was observed. The lining epithelium was composed of cuboidal/columnar cells with mild atypia, with micropapillary extensions. Immunohistochemistry revealed double-layered neoplastic epithelium containing two different types of cells: basaloid/myoepithelial and luminal. We recommend two out of vimentin, HMWCK, and D2-40 for myoepithelial/basaloid cells, also CK7 and EMA for luminal/columnar cells. As the tumor had infiltrated the surgical margins, the patient underwent axillary sentinel lymph node (SLN) dissection and re-excision with Mohs micrographic surgery (MMS). Two additional MMS stages were required due to suspicious surgical margin positivity in the frozen sections. The operation was continued despite the risk of loss of function. Upon examination of the permanent sections, we observed no tumors in the suspected positive foci. Additionally, no tumor was found in the surgical margins. No metastasis was detected in the sentinel lymph node. We have reached 300 reported cases of DPA in the literature. We discussed the histopathological and intraoperative diagnostic pitfalls of DPA with a literature review and our experience.