History:

A 20-year-old female suffered various manifestations of neurofibromatosis-1 since early childhood. At age of 12 years a large plexiform neurofibroma had been surgically removed from the upper arm (see large wide scar in first two photos). However, another giant plexiform neurofibroma just below the first one was still evident at time of presentation. Cafe-au-lait pigmentation, axillary freckling in addition to other defects (nevoid hyperpigmentation and hypertrichosis on the face) were seen.