A 19-year-old man presented with multiple asymptomatic lobulated and cerebriform papulonodules and plaques over his left lower trunk that had been present since childhood. The lesions first appeared at the age of 1 month as several tiny red macules over his left lower back and then gradually spread to his abdomen in a linear fashion and became elevated. The lesions progressed more rapidly during his early teens and then remained stationary. There was no relevant family history. A physical examination revealed many soft, erythematous to slightly brownish papules and nodules coalescing into pebbly plaques and soft, cerebriform, polypoid tumors over the left lower trunk in a linear pattern ( Figure 1A and B). Most of the papules had a central umbilication or hair follicular openings ( Figure 1C). There were also some keratinous plugs intermingled with the nodular lesions. All other physical and laboratory examinations were normal. A shave biopsy was performed on a papule ( Figure 1B, arrow) and showed a fibrocollagenous polyp with vertical or intersecting collagen bundles and many small blood vessels in the dermis (Figure 2A), where small clusters of mature fat cells were found around the blood vessels (Figure 2A, inset). In addition, there were a few sebaceous lobules and eccrine glands in the polyp. The findings were suggestive of a hamartoma with epithelial and mesenchymal components. He then underwent curettage and carbon dioxide laser treatment for the complete removal of the skin lesions. Repeated histopathological examination revealed fibrocollagenous polypoid lesions with the presence of sweat glands, sweat ducts, pilosebaceous units ( Figure 2B), and perivascular fat cells within the polyps. The scars remained flat with a few small residual keratinous plugs and draining fistulas eight years after the operation.The clinical and histopathological presentations of this patient were characterized as follows: (1) the skin lesions consisted of umbilicated papules with coalescence into pebbly plaques and large soft polypoid tumors with a cerebriform surface; (2) the lesions showed a distinct linear and blaschkoid pattern; and (3) histology showed polypoid fibrocollagenous lesions with multiple types of epithelial adnexal structures (pilosebaceous and sweat glands) and ectopic adipocytes around superficial blood vessels. The constellation of these findings appears to be unique.Cutaneous polypoid hamartomas with fibrocollagenous stroma in a linear or zosteriform distribution can be seen in the classical form of nevus lipomatosus superficialis (NLS). NLS is categorized into the classical form (HoffmanneZurhelle) and the solitary form. 1 The solitary form has been regarded as a pedunculated lipofibroma. 2 The classical form usually occurs at birth or within the first two decades of life and manifests as skin-colored to yellowish, peau d'orange, cerebriform, or smooth-surfaced papulonodules, often coalescing into segmental or zosteriform plaques. 1 The most common locations are the pelvic girdle, abdomen, back, buttock...