Many studies concluded that the aberrant HH signaling pathway is active in the development of OKCs.215,216 Grachtchouk et al. uncovered that elevated expression of HH target genes is detected in lower cell layers of cyst wall of human OKCs.217 Besides, OKCs were found to express lower levels of Sufu gene and higher level of Smo, Ptch1, Cyclin D1 and Bcl2.218 Syndromic OKCs were found to have a higher expression of HH signaling pathway protein than the sporadic OKCs.219 Higher Smo expression was considered to be a risk factor of recurrence of OKCs.220. Here, SMO is linked to cyst.