Background: Clinically, folliculosebaceous cystic hamartomas (FSCH) pr
esent as skin-colored papulonodules of the face, especially the nose.
This is in contrast to sebaceous trichofolliculomas which often presen
t as depressed ostia containing terminal or vellus hairs. Observation:
A 34-year-old female had a skin-colored multinodular plaque of the la
bia majora that demonstrated the histologic findings of an FSCH. These
included an irregularly shaped central cystic structure surrounded by
multiple sebaceous lobules as well as a fibrous stroma. The epithelia
l-lined cyst contained no hair shafts. Conclusion: A single case repor
t of a scrotal lesion with a similar clinical appearance and histologi
c findings has previously been described as a genital variant of sebac
eous trichofolliculoma. However, the report predated the initial descr
iption of FSCH. In our opinion, both cases represent examples of the g
enital variant of FSCH.