Abstract: Perianal Paget's disease (PPD) is a type of extra-mammary Paget disease, and its essence is intrepithelial adenocarcinoma of the skin.The etiology and pathogenesis of PPD remain unclear,and its clinical features resemble non-malignant inflammatory disorders,often leading to delayed diagnosis and treatment.This report describes a rare case of primary perianal Paget's disease.The patient underwent local wide excision combined with modified V-Y flap reconstruction,achieving good postoperative recovery.

Key words: perianal Paget's disease; local excision; flap reconstruction