Abstract Secondary extramammary Paget’s disease (EMPD), originating from the cutaneous dissemination of malignant cells, represents a rare subtype prone to misdiagnosis. This study presents a case of secondary perianal Paget’s disease (PPD) associated with adenocarcinoma at the anorectal junction. Preoperative full-thickness mapping biopsy was utilized for the assessment of lesion margins. The patient demonstrated favorable postoperative recovery following a multimodal therapeutic regimen.