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Cutaneous endometriosis is a rare condition but represents the most common extrapelvic location. Primary cutaneous endometriosis may develop in the umbilicus, perianal region or inguinal region. We report the case of a female patient with a diagnosis of Villar’s nodule that was successfully treated with surgery. The pathophysiology of this entity is not well defined yet. The clinical suspicion is fundamental for the diagnosis. Complementary imaging test are used to rule out the differential diagnoses. Surgery is indicated in all the cases. The prognosis is favorable in most cases, with low rate of recurrence.