Adenocarcinoma of the anal glands is a rare slow-growing tumor with a more favorable prognosis compared with colorectal adenocarcinoma, especially if an early diagnosis is established. Clinical symptoms of this disease, often associated with a fistula in ano as in the reported case, include: perianal pain, rectal bleeding and presence of perianal mass. Also perianal Paget's disease may be a not rare association with adenocarcinoma of the anal glands. We believe, therefore, that a histological examination of the resected fistulas in ano should be performed, in searching for the presence of mucinous granules. In addition, it's very important to carry out a careful examination of those patients presenting pruritus ani or eczematous lesions of the anal region. In fact adenocarcinoma of the anal glands may be due to a chronic irritation of the epithelium over a period of years. Radiation therapy and chemotherapy have proved not to provide survival benefit in the treatment of this disease; the same result is obtained with a local excision of the lesion. The only chance for cure, therefore, is early diagnosis followed by radical operation. Miles abdominoperineal resection represents the approach of choice we have adopted for our patient. A radical groin dissection should be carried out only if there are metastases to the inguinal nodes. If necessary, abdomino-perineal resection may be folowed by adjuvant irradiation.