Nevus lipomatosus cutaneous superficialis is a rare idiopathic hamartomatous anomaly of the skin characterized by the presence of clusters of mature fat cells among the collagen bundles of the dermis. The classic, or solitary type, presents with asymptomatic soft, skin-colored to yellow papules or nodules. We report the case of a 12-year-old boy with congenital, classical nevus lipomatosus cutaneous superficialis that started as a single painless nodule. Over time, the nodule developed into lesions that presented as skin-colored, well-defined, soft sessile growths with a cerebriform surface centered by comedo-like plugs that increased in size and spread over a large area (approximately 12×4 cm) over the right gluteal region. We report this case as it is an uncommon condition with the intent to highlight its clinical and histopathological features, and differential diagnosis.