Endometriosis is defined as the presence and growth of the glands and stroma of the lining of the uterus in an aberrant or heterotopic location. The cause of endometriosis is uncertain and involves many mechanisms including retrograde menstruation, vascular dissemination, metaplasia, immunologic, and hormonal influences to name a few. Primary umbilical endometriosis is a rare disorder defined as the presence of ectopic endometrial tissue within the umbilicus. It commonly presents with catamenial pain and bleeding from an umbilical nodule. It is a rare condition and treatment has not been standardized yet due to the limited number of cases. Surgical management is the treatment of choice to avoid local recurrence while hormone therapy may be used preoperatively for symptomatic relief. This paper reports a series of recently observed cases of primary umbilical endometriosis with main aim of discussing the different approaches in the management of this rare condition.