We report the case of a patient diagnosed with primary umbilical endometriosis intending to discuss the diagnostic and therapeutic management of this rare disease. A 45-year-old woman suffering from a painful swelling located in the umbilical region, with intact and normal cutaneous aspect, came to our attention. Ultrasonography of the umbilical region showed a nodule with a nonhomogeneous echotexture pattern. Partial omphalectomy was performed under local anesthesia in day care setting surgery. Histology confirmed the diagnosis of umbilical endometriosis. Pre- and postoperative clinical controls showed no evidence for other endometriosis localization. No medical treatment was administered. No signs of recurrence were observed after 5 years from surgery. A review of the literature of the last 10 years was generated based on MEDLINE research, selecting some specific keywords. Several lesions can occur in the umbilical region, and endometriosis has to be ruled out even in patients without any surgery in their medical history. Surgery is the gold standard treatment for this condition: partial and radical omphalectomy are the two treatment options. We believe that given the significant psychological and aesthetical value of the umbilicus, surgical treatment has to be tailored and in case of a small endometrial umbilical nodule, partial omphalectomy (local excision of the umbilical endometrial nodule) with a 3 mm free border, even without adjuvant hormonal treatment, could ensure adequate and effective treatment.