Endometriosis refers to the existence of endometrial tissue outside the uterus. This is a non-threatening illness that impacts 10-15% of women [1]. It primarily affects women who are in their reproductive years. The lesions primarily manifest in pelvic locations, specifically the ovaries, uterosacral ligaments, ovarian fossa, cul-de-sac, and bladder, in that sequence [2]. Extrapelvic endometriosis is a less frequent occurrence. The extrapelvic locations encompass the diaphragm, pulmonary system, urinary tract, gastrointestinal tract, brain, and cutaneous endometriosis. Umbilical endometriosis is a rare condition, accounting for only 0.5-1% of all cases of extrapelvic illness. Umbilical endometriosis, albeit uncommon, is the most prevalent form of cutaneous endometriosis [3]. Umbilical endometriosis can be classified as primary if it occurs spontaneously, or secondary if it happens after laparoscopic or surgical surgeries. The latter is more frequently observed [4]. Villar's nodule, commonly referred to as primary umbilical endometriosis, was initially documented by Villar in 1886 [5]. The etiology of endometriosis is poorly comprehended. Theories that have been proposed include Sampson's idea of retrograde menstruation, which is the most prevalent, coelomic metaplasia, induction theory, embryonic Mullerian resting, bone marrow stem cell theory, and hematogenous/lymphatic spread. In cases of umbilical endometriosis, the most favored idea is that it spreads through the blood or lymphatic system, particularly when there is also pelvic endometriosis present. It is also possible for isolated umbilical endometriosis to originate from the transformation of urachal remains. The objective of this study was to assess the clinical features, manifestation, diagnosis, and treatment of umbilical endometriosis based on existing literature.