Lannelongue and Menard were the first to report this rare entity in 1891  and since then less than 100 cases have been recorded in literature. The pathogenesis of midline upper lip fistulae is still debatable and a number of hypotheses have been proposed on this issue. Some have proposed that the early or abnormal epithelial inclusion events may occur in the medial fusion area during formation of the intermaxillary process [2,4,5] while others have suggested abnormal fusion of facial prominences or merging of mesoblasts . The upper lip fistulae are seldom associated with other malformations ; however if such co-anomalies occur, they are midline malformations, such as double frenulum, medial cleft, nasal dermoid or hypertelorism .
Management comprises of complete surgical excision; incomplete excision leads to recurrences and ultimately cosmetic deformities. Based on our previous experiences of management of fistulae in other areas of body, we injected 0.5% methylene blue through the cutaneous mouth of fistula to facilitate the complete excision as entry into the fistulous tract would have stained the tissues and served as indicator of wrong plane of dissection.