Primary extramammary Paget's disease is a rare intra-epidermal neoplasm originating from apocrine gland duct cells or keratinocyte stem cells. Clinically, it manifests as asymmetrical white and red plaques, leading to frequent diagnostic delays due to nonspecific features. This prospective study, conducted from September 2021 to July 2023, included twenty cases with confirmed histopathology. Patients had a mean age of 69.25 years with a slight male predominance (55%). Vulva (40%) and scrotum (30%) were common initial sites in females and males, respectively. Paget cells were universally present, with acantholysis in 20% of cases. In situ disease occurred in 80%, while dermal invasion was seen in 20%, with hair follicles most commonly involved. This study underscores the importance of recognizing adnexal involvement in extramammary Paget's disease, aiding in accurate diagnosis and management.