Pilonidal sinus disease is seen most commonly in the sacro-coccigeal area. Umbilical pilonidal sinus is a rare form of the condition with few studies about its clinical course and management. The current review is to highlight the presentation and management of umbilical pilonidal sinus. Literature review revealed 25 papers which contained 385 patients complaining from umbilical pilonidal sinus. Chronic discharging sinus and pain were the two most common presenting symptoms. Both conservative and surgical management were effective methods of therapy.