Congenital midline labial sinus is a rare condition known to affect both the upper and the lower lip. This anomaly has been reported in both genders, but with a slightly increased predilection in females. It can occur either in isolation or in association with a wide array of congenital malformations such as cleft lip, cleft palate, Van der Woude syndrome, median lip pit, and brachydactylia. Usually, the patient presents with swelling of the upper lip with or without associated discharge. A history of recurrent cellulitis of the lip is almost always present. In this manuscript, a review of the literature (spanning across almost six decades) has been attempted for this rare condition. Search engines such as PubMed, Medscape, and Google Scholar were searched for keywords such as “recurrent cellulitis of the upper lip,” “sinus of the upper lip,” “fistula of the upper lip,” and “congenital midline labial sinus.” All the available full text in the English language was compiled and thoroughly scrutinized. The observations such as age, sex, clinical feature, associated anomaly, and other relevant information were noted and tabulated in the chronological order.