ABSTRACT Primary extramammary Paget's disease (EMPD) is a rare intra-epidermal neoplasm originating from apocrine gland duct cells or pluripotent keratinocyte stem cells. It clinically manifests as asymmetrical white and red plaques, often leading to delayed diagnosis due to its rarity and non-specific features. This study aimed to elucidate the clinical and pathological characteristics, particularly focusing on adnexal involvement patterns. Twenty cases were prospectively analyzed from September 2021 to July 2023. A slight male predominance was noted, with vulva and scrotum being the most commonly affected areas in females and males, respectively. Histopathological analysis revealed Paget cells as a universal feature, with in situ disease observed in 80% of cases and dermal invasion in 20%. Adnexal involvement was common, notably with hair follicles being the most frequently affected structure. This study underscores the significance of recognizing adnexal involvement in primary EMPD, with implications for diagnosis and treatment planning. Keywords: EMPD; Adnexa; Histopathology