Nevus lipomatosus cutaneous superficialis is a very rare skin disease which usually is present from birth. This uncommon condition is due to collections of ectopic. fat cells within the upper and mid dermis. There are two clinical varieties. The first is a lesion of zonal distribution, present from birth or childhood, usualIy on the buttocks or the lower back. The second form, a dome or sessile, papule, begins. in adult life and is less restricted in distribution. Cerebriform plaques tend to form from the coalescence of soft, yellowish papules. The authors observed two cases of typical nevus lipomatosus cutaneous superficialis. The first case is a 25-year-old female who has had asymptomatic, soft, skin colored, and huge confluent nodules on the right infragluteal fold and upper thigh for 15 years; and the second case is a 15-year-old male who has had multiple, skin colored and soft papules or nodules in zosteriform distribution affecting the right side of the lower back and coccygeal area and right buttock for 4 years. The authors made the diagnosis of nevus lipomatosus cutaneous superficialis from the characteristic clinical and histopathological findings.