ABSTRACT
Giant congenital melanocytic nevi (GCMN) are the melanocytic lesions that arise due to the abnormal migration of melanoblasts during the embryogenesis, affecting approximately one in 500,000 live births. There is gain-of-function mutation in the NRAS gene is usually associated with GCMN, causing abnormal proliferation of embryonic melanoblasts that are usually present since birth with change in their morphological characteristics with time, and increasing in their size reaching upto a diameter ? 20 cm in adulthood. These are characterized by dark brown to black in color with irregular margins and having verrucous surfaces, with or without satellite lesions which are present beyond the periphery of the central lesion. Depending on their distribution these can be classified as bathing trunk, coat-sleeve, or stocking nevi. We hereby report a case of a sixteen year old female presenting with GCMN. The development of a malignant neoplasm on same lesion is only absolute indication for surgery in GCMN