ABSTRACT

Giant congenital melanocytic nevi originate from melanocytes. It is usually seen after birth and often found in dermis and rarely spreads through subcutaneous tissue. Giant melanocytic nevi have a greater diameter than 20 cm, dark in color and consist of a lot of melanocytes. Giant congenital melanocytic nevi patients should be under long term follow-up because of their malignant potential. We report a two year-old girl presenting with a GCMN which involves % 80 of body surface. Lesion begins under nipples and elongates through symphysis pubis spares the midline in front of the body, and on the back; beginning from the servical region elongates through 1/3 of upper thigh. She was noted arachnoid cyst in brain and atrophy of 1.metacarpal and phalangial bones on the left hand while searching for any other abnormalities. We present this case for involving skin, bone frame and brain.