Endometriosis is a gynecologic condition affecting mainly the pelvic organs. However, extrapelvic endometriosis has been reported in almost all parts of the body. Umbilical endometriosis, either primary or secondary, is uncommon and has a documented neoplastic risk. We present the case of a 46-year-old woman with a large umbilical hernia associating primary umbilical endometriosis discovered during surgery and confirmed later by pathological and immunohistochemical exams. The patient underwent omphalectomy and partial omentum resection, alongside with mesh abdominal wall repair. The patient was informed about the recurrence risk and was asymptomatic at follow-up consults.