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This article discusses the successful treatment of epidermal nevus (EN) in a patient with phacomatosis spilosebacea (PSS) using topical sirolimus. EN is a type of birthmark, while PSS is a rare disorder characterized by the presence of EN along with other organ system involvement. Treatment of EN can be challenging, but topical sirolimus has shown promising results in managing difficult-to-treat dermatological disorders. In this case, a patient with PSS experienced a marked decrease in thickness of the EN lesions after 5 weeks of treatment with topical sirolimus, with no adverse effects reported. Further follow-up is needed to confirm the long-term improvement. [Extracted from the article]