Introduction: We report a recently observed case of secondary umbilical endometriosis (UE), with the main aim to discuss diagnosis and the management of this rare condition. Observation: A 41-year-old woman admitted to our facility with a nodular, bluish umbilical swelling, non- painful, gradually increasing in size, bleeding with her menstrual cycle. Ultrasonography did not reveal any images indicative of pelvic endometriotic lesions. A pelvic MRI was performed wich showed an ovoid formation at the umbilical level, suggestive of a UE implant. A biopsy of the lesion was also carried out, showing a morphological appearance compatible with parietal endometriosis. Histopathological analysis confirmed the diagnosis of UE. Neither symptoms or signs of local recurrence have been observed after 6 months. Discussion: Extra-pelvic endometriosis sites are not common, especially the umbilicus. It usually occurs secondary to surgical scars, specifically after laparoscopy or open abdominal surgery. UE should be taken into accounting differential diagnosis of umbilical disorders even in young nulliparous women with no typical symptoms of pelvic endometriosis. UE can be treated medically or surgically. Surgical treatment options are either local excision of the lesion or removal of the whole umbilicus with or without laparoscopic exploration of the peritoneal cavity. The decision should be tailored for the individual patient, taking into consideration the size of the lesion, the duration of symptoms and the presence of possible pelvic endometriosis. Conclusion: Local excision saving the umbilicus may be the treatment of choice in patients with small UE lesions.