Giant Congenital Melanocytic Nevus with Limb Hypoplasia and Lipomatosis: ARare Association

The congenital melanocytic nevi (CMN) are very rare, hyper pigmented macular lesions formed by overgrowth of the melanocytes and melanoblasts. This exists since birth and also named as bathing trunk, coat sleeve or stocking nevi. They occur in less than 1% of the neonates in any site of the body but commonly occur over the trunk and thigh areas. The giant congenital nevus is greater than 20 cm in size, pigmented and often hairy. Here, we are discussing a classical case of large congenital melanocytic nevus presented over right part of torso, lower limb, lower back and buttocks, with multiple satellite lesions all over body with right lower limb hypotrophy. The diagnosis was confirmed by histopathology. Apart from the rarity of the disease, this case report highlights the rare association of CMN with hypotrophy of limb.