Objective: This report presents a rare case of symptomatic primary umbilical endometriosis and reviews the literature on the topic with the aim to clarify some questions on the origin of endometriosis. Case Report: A 33-year-old woman with cyclic umbilical bleeding was found to have umbilical endometriosis. She had no history of pelvic or abdominal surgery. There was no past history of endometriosis or endometriosis-associated symptoms. An omphalectomy was performed after explorative laparoscopy to carefully inspect the abdominopelvic cavity and assess any coexisting pelvic endometriotic lesions. Histological examination confirmed the diagnosis of umbilical endometriosis. Conclusion: Umbilical endometriosis is a rare but under-recognized phenomenon. Primary lesions are difficult to recognize, but probably represent an independent nosological entity. The possibility of endometriosis must be considered during the evaluation of an umbilical mass despite the absence of previous surgery. Complete excision and successive histology are highly recommended.