Nevus Melanositik sejak lahir ditemukan pada satu persen anak di dunia dengan ukuran bervariasi. Salah satu varian nevus melanositik yang sangat jarang, berukuran sangat besar, yakni Nevus Melanositik Kongenital Raksasa (NMKR). Pada tulisan ini dilaporkan kasus NMKR disertai kelainan kongenital ekstremitas. Seorang bayi perempuan lahir di puskesmas dengan partus spontan dirujuk ke IGD, 3-4 jam setelah lahir. Terdapat bercak hitam di area perut, punggung, dan paha. Didapatkan juga perbedaan panjang tungkai, serta hanya ada 3 jari di kaki kanan. Tatalaksana diberi pelembap. Kasus ini jarang; diperlukan konsultasi dan observasi teratur untuk meningkatkan dan memperpanjang harapan hidup.Melanocytic Nevus since birth is found in one percent of children in the world with variable severity. One of the rare variant is very large melanocytic nevus variant called Giant Congenital Melanocytic Nevus (GCMN). This paper reported a case of GCNM accompanied with congenital abnormalities of the extremities. A baby girl who was born spontaneously in a clinic, was referred to the emergency department about 3-4 hours after her birth. The abnormalities were black spots in the abdominal area, back, and thighs. The patient also had different length of legs differences with only 3 toes on right foot. The patient was given moisturizer. GCMN case accompanied with congenital limb abnormalities is rare; regular consultation and observation are required to improve and prolong survival.
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