[Progressive circumscript lentiginosis--phakomatosis pigmentovascularis III/A]

The case of a young woman with circumscribed lentiginosis on certain parts of her body with nevi flammei on face, neck and trunk is reported. This patient's condition was significant in two ways: she had a progressive circumscribed lentiginosis or lentiginosis syndrome, and it was associated with lentigo, nevus spilus and nevus flammeus, so that it was suggestive of phakomatosis.