Extramammary Paget's disease (EMPD) is a rare, malignant skin disorder primarily affecting areas rich in skin appendages, such as the vulva, genital areas, and axillary skin. It presents as erythematous skin lesions resembling eczema, typically progressing slowly. EMPD can be primary or secondary, with primary EMPD originating as an intraepithelial neoplasm of the epidermis. Adnexal involvement, particularly in hair follicles and ecrine ducts, is common in primary EMPD, though its correlation with tumor progression or recurrence remains inconclusive. Surgical excision, including Mohs micrographic surgery, is the primary treatment, with topical agents and systemic treatments used in advanced cases. Future research is needed to better understand adnexal involvement's depth and significance to design effective targeted therapies. This systematic review aimed to evaluate the significance of adnexal involvement and depth in primary EMPD concerning recurrence and prognosis. A comprehensive search of multiple databases identified 195 articles, with 9 eligible for inclusion. The review found that over 90% of primary EMPD cases exhibit adnexal involvement, predominantly in hair follicles and ecrine ducts. While adnexal involvement does not significantly correlate with tumor progression or recurrence, it may facilitate deeper tissue infiltration, highlighting its clinical importance. Therapeutic options for primary EMPD range from topical treatments like imiquimod and 5-fluorouracil to surgical excision. Mohs micrographic surgery is preferred for its enhanced margin control. The efficacy of topical agents depends on the depth of Paget cell invasion within adnexal structures, emphasizing the need to understand the pattern and extent of this invasion to optimize patient management. For metastatic disease, systemic treatments such as 5-fluorouracil and cisplatin are used, though prognosis remains poor. Future directions include developing targeted therapies and improving diagnostic modalities to better address adnexal proliferation in primary EMPD.Extramammary Paget's disease (EMPD) is a rare, malignant skin disorder primarily affecting areas rich in skin appendages, such as the vulva, genital areas, and axillary skin. It presents as erythematous skin lesions resembling eczema, typically progressing slowly. EMPD can be primary or secondary, with primary EMPD originating as an intraepithelial neoplasm of the epidermis. Adnexal involvement, particularly in hair follicles and ecrine ducts, is common in primary EMPD, though its correlation with tumor progression or recurrence remains inconclusive. Surgical excision, including Mohs micrographic surgery, is the primary treatment, with topical agents and systemic treatments used in advanced cases. Future research is needed to better understand adnexal involvement's depth and significance to design effective targeted therapies. This systematic review aimed to evaluate the significance of adnexal involvement and depth in primary EMPD concerning recurrence and prognosis. A comprehensive search of multiple databases identified 195 articles, with 9 eligible for inclusion. The review found that over 90% of primary EMPD cases exhibit adnexal involvement, predominantly in hair follicles and ecrine ducts. While adnexal involvement does not significantly correlate with tumor progression or recurrence, it may facilitate deeper tissue infiltration, highlighting its clinical importance. Therapeutic options for primary EMPD range from topical treatments like imiquimod and 5-fluorouracil to surgical excision. Mohs micrographic surgery is preferred for its enhanced margin control. The efficacy of topical agents depends on the depth of Paget cell invasion within adnexal structures, emphasizing the need to understand the pattern and extent of this invasion to optimize patient management. For metastatic disease, systemic treatments such as 5-fluorouracil and cisplatin are used, though prognosis remains poor. Future directions include developing targeted therapies and improving diagnostic modalities to better address adnexal proliferation in primary EMPD.