Perianal Paget's disease (PPD) is part of the spectrum of pagetoid skin lesions occurring outside the region of the nipple/areolar complex that are collectively referred to as extramammary Paget's disease (EMPD). However, unlike Paget's disease of the breast, which in the vast majority of cases is invariably associated with an underlying ductal carcinoma, most cases of EMPD occur as either insitu or invasive adenocarcinomas with visceral malignancy being a rare entity. We present a case of a 50-year-old man who was referred to us with PPD. This case highlights the difficulties associated with making a clinical diagnosis of this condition. Furthermore, investigative workup to exclude possible malignancy with associated metastatic spread as well as the treatment options available are also discussed. In addition, this case highlights the need for a multidisciplinary team approach when dealing with this difficult problem.