Extramammary Paget’s disease (EMPD) is an uncommon rare intraepithelial neoplasm that affects apocrine gland-bearing skin. The typical histologic features of EMPD include Paget’s cells with hyperchromatic nuclei and pale cytoplasm. However, sometimes it can be challenging to differentiate EMPD with other skin neoplasm showing pagetoid growth pattern such as pagetoid melanoma and pagetoid squamous cell carcinoma in situ, especially when showing atypical histologic morphology such as an cantholysis with full-thickness atypia. A 83-year-old men presented with pricking, diffuse, erythematous erosive patch on the scrotum for 20 years. The skin lesion was not responsive to any previous treatment including topical steroid or antifungal agent. Histopathologic findings revealed acanthosis in epidermis with pagetoid cells. There was also prominent acantholytic epidermis with full thickness atypia containing pleomorphic cells. Immunohistochemical staining showed that cytokeratin 7 (CK7), carcinoembryonic antigen (CEA) were strongly expressed in nests of atypical large tumor cells but cytokeratin 20 (CK20) were negative, which was compatible with EMPD. The patient was diagnosed with Acantholytic extrammammary paget’s disease and is being treated with cryotherapy. Herein, we report a case of acantholytic extramammary Paget’s disease which was rarely reported and immunohistochemical findings can be useful in such a situation.