Congenital Giant Melanocytic Nevus (CGMN), pigmented lesion present since birth, occurs in 1 % of infants worldwide. Fifteen percent of CGMN are localized in the head and neck region and it can also have a bathing trunk distribution. It grows proportionally to the size of the body as the child matures and grows with variation in colour and surface texture. A 29 years old female presented to the Gynecology Out Patient Department for infertility. She also had multiple large nevi of varying sizes present since birth. The lesion was distributed all over the body. She also complained of sudden appearance of swellings at the back which were later excised and the histopathological examination showed the presence of neural nevus involving the dermis and subcutaneous tissue. This case is being reported for its rarity, the higher risk for melanoma transformation, its association with meningeal melanosis and few benign / malignant tumors.
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