Extramammary Paget’s Disease (EMPD) is a rare and aggressive skin disorder primarily affecting areas rich in hair follicles, with the vulva being the most common site. EMPD presents as erythematous skin lesions resembling eczema and can progress slowly, either as primary or secondary manifestations. Primary EMPD originates as an intraepithelial neoplasm of the epidermis, often leading to local and distant metastases. This systematic review aims to evaluate the significance of adnexal involvement and depth in primary EMPD concerning recurrence and prognosis. The review included studies focusing on EMPD, adnexal involvement, depth, recurrence, and prognosis. Adnexal involvement, particularly in hair follicles and eccrine ducts, is prevalent in primary EMPD, but its correlation with tumor progression or recurrence rates remains inconclusive. Surgical excision, including Mohs micrographic surgery, is the primary therapeutic approach, while topical agents and systemic treatments are used in advanced cases. Future studies are essential to understand the depth and significance of adnexal involvement to design effective targeted therapeutic approaches in EMPD. The prognosis and recurrence of primary EMPD are closely linked to the extent of adnexal involvement, highlighting its clinical importance. Therapeutic options range from topical treatments to surgical excision, with Mohs micrographic surgery being preferred for its enhanced margin control. Understanding the pattern and extent of Paget cell invasion within adnexal structures is crucial for optimizing patient management strategies.Extramammary Paget’s Disease (EMPD) is a rare and aggressive skin disorder primarily affecting areas rich in hair follicles, with the vulva being the most common site. EMPD presents as erythematous skin lesions resembling eczema and can progress slowly, either as primary or secondary manifestations. Primary EMPD originates as an intraepithelial neoplasm of the epidermis, often leading to local and distant metastases. This systematic review aims to evaluate the significance of adnexal involvement and depth in primary EMPD concerning recurrence and prognosis. The review included studies focusing on EMPD, adnexal involvement, depth, recurrence, and prognosis. Adnexal involvement, particularly in hair follicles and eccrine ducts, is prevalent in primary EMPD, but its correlation with tumor progression or recurrence rates remains inconclusive. Surgical excision, including Mohs micrographic surgery, is the primary therapeutic approach, while topical agents and systemic treatments are used in advanced cases. Future studies are essential to understand the depth and significance of adnexal involvement to design effective targeted therapeutic approaches in EMPD. The prognosis and recurrence of primary EMPD are closely linked to the extent of adnexal involvement, highlighting its clinical importance. Therapeutic options range from topical treatments to surgical excision, with Mohs micrographic surgery being preferred for its enhanced margin control. Understanding the pattern and extent of Paget cell invasion within adnexal structures is crucial for optimizing patient management strategies.