Café-au-lait neurofibroma, also known as neurofibroma café-au-lait, is commonly found in type I neurofibroma. It is mainly due to genetic defects leading to abnormal development of nerve cells, thus causing multi-system and multi-organ damage. Neurofibroma cafe au lait spots are commonly found on the skin surface with folds such as armpits, limbs, trunk, etc. The color of cafe au lait spots is brown with different shades of coffee, and the shape of cafe au lait spots is willow-leaf or oval with different sizes. If the number of coffee spots is greater than 6, and the diameter is greater than 5mm before puberty, and the diameter is greater than 15mm after puberty, we should be alert to the possibility of developing into neurofibromatosis. It is recommended that patients should be diagnosed and treated early if symptoms appear, so as not to delay the condition.