We report a rare case of primitive umbilical endometriosis associated with umbilical hernia, which required wide umbilical resection and immediate reconstruction performed according to a recently described technique. A wide resection of the peritoneal sac was performed because of the possible presence of endometrial tissue inside. The hernia was repaired according to Mayo's technique. Umbilical reconstruction was performed using two semicircular skin flaps shaped into in an ellipse preoperatively drawn on the skin with a vertical orientation. The surgical approach described in this report allowed easy hernia repair and umbilical reconstruction. No prosthesis was used because of the small size of the hernia and lack of literature data on prosthesis use in endometriosis. The aesthetic result was considered satisfactory 6 months after the operation because of absence of hypertrophic scars and in view of the anatomical aspect of the new umbilicus. No recurrence was observed within this time frame.