Introduction: Frontorhiny is a type of frontonasal malformation that also known as median facial cleft syndrome characterised by hypertelorism, wide nasal bridge, short nasal ridge,splayed nasal bone, bifid nasal tip, widened columellar, long philtrum and midline notch In the upper lip. This sporadic congenital disorder is a rare autosomal recessive caused by homozygous mutations of ALX 3 gene, which is important in facial embryogenesis. There were 42 cases reported worldwide from 1980 to 2009, mainly from Brazil (10 cases), followed by London (5 cases), Bahamas (4 cases) and Venezuela (3 cases).
Case report: We present the first extremely rare case of frontorhiny in Malaysia, highlighting the clinical features and the surgical outcome.
Conclusion: Frontorhiny, ALX-3 related FNM,is an extremely rare frontonasal malformation with typical clinical features that presents a surgical reconstructive challenge and some cases may need multi stage surgery.