Paget's disease (PD) of the anus is a rare perianal disorder. The condition is often associated with underlying invasive carcinoma, and the prognosis is poor when underlying adenocarcinoma is present.

We report the case of a 72-year-old woman suffering from a 2-year history of perianal itching, bleeding and irritation treated with a variety of ineffective topical agents before the diagnosis of perianal PD (PAPD) was confirmed by histopathologic demonstration of Paget's cells from a biopsy. Radiological and further clinical evaluation allowed exclusion of any synchronous malignancy or metastasis.

A first-step surgery consisted of a wide excisional biopsy with frozen section control of margins. Permanent sections confirmed absence of invasive carcinoma and clear margins. Four days after wide excision, bilateral gluteal flap reconstruction was performed with temporary diverting colostomy and Hartmann pouch. No adjuvant therapy was necessary, and after one-year follow-up, the patient remains disease-free.

Our case report of PAPD stresses the importance of diagnosis to try and improve the poor prognosis of this rare disease, and shows one method of management for advanced non-invasive disease.