The numerous hypotheses advanced to explain the pathogenesis of lip sinuses have been reviewed by Warbrick et al. (1952) who suggested that the sinuses are remnants of the lateral sulci of the lower lip at the 6.5512.6mm embryonal stage (32-40 days). This theory is now generally accepted by such authorities as Gorlin et ul. (1976) and Wang and Macomber (1977). The sinuses usually appear together with or in families who present with various types of clefts. This distinct clinical syndrome is thought to be transmitted through an autosomal dominant gene with an estimated penetrance of 800,” and variable expressivity (Cervenka et al., 1967). Lip sinuses have also been described in the popliteal pterygium syndrome (Rintala and Lahti, 1970b) and occasionally with other malformations such as Hirschprung’s disease (Schwartz et aI., 1979). Over 500 cases have been reported in the literature and the incidence of the syndrome has been estimated as 1: 100,000 in the general white population (Cervenka et al., 1967) and as 0.9 (j,,, in patients who have cleft lip and/or cleft palate (Rintala and Lahti, 1970a). The typical sinuses are bilateral symmetrically placed openings on the upper border of the lower lip which extend as canals lined by labial mucosa and surrounded by mucous glands towards the labio-gingival sulcus for a varying distance of 1 to 25mm. The canals always end as blind sacs. Sometimes there may be only conical elevations and/or surface openings without any deeper sinuses at the typical sites. presumably representing microforms of the syndrome and very rarely unilateral or median sinuses may be encountered. Some of the latter are bipartite in their deeper parts (Rintala and Lahti, 1973) and in some it is difficult to decide whether they are truly median or paramedian. Their location may also vary in an anterio-posterior direction from the vermilion border to the mucosal side of the lip.