Extramammary Paget's disease (EMPD) is a rare malignancy primarily affecting apocrine gland-bearing areas such as the vulva, scrotum, and penis, with limited treatment data due to its infrequency. This review, based on literature up to February 2022, outlines a contemporary approach to diagnosing and managing EMPD. Typically presenting as an indolent carcinoma in situ, the cornerstone of treatment is prompt and accurate diagnosis followed by wide local or Mohs micrographic surgical excision, with careful management of margin status and consideration of lymphadenectomy for regionally positive disease. While conventional chemotherapies serve as alternatives for patients with distant metastases, their efficacy can be suboptimal, and consensus on adjuvant or systemic therapies is lacking. Recent advances in understanding the molecular pathogenesis and genomics of EMPD may pave the way for targeted treatments, underscoring the importance of specialized care, vigilant follow-up, and global collaborative efforts to design robust clinical trials for this orphan disease.