Woolly hair nevus (WHN) is a rare hair condition, with only a few cases reported. It affects young children of both sexes and currently, there is no available treatment. When WHN is associated with an epidermal nevus, further study is required to rule out ophthalmologic and neurologic abnormalities. We present the case of a Chilean 3-year-old healthy girl with a WHN, with no other clinical findings. This case illustrates a WHN in which the benign and chronic nature of the disease must be advised to the parents in order to prevent further concerns.