Abstract :
Microcephalic osteodysplastic primordial dwarfism(MOPD) is a rare autosomal recessive disorder characterised by severe cause of microcephaly, facial dysmorphism, central nervous system , skeletal,skin and limb abnormalities. Recently mutations in RNU4atac SnRNA ,have been recognised as the cause of MOPD. Less than 50 cases have been reported worldwide.We are reporting a case of a 18week- old female fetusdiagnosed with MOPD.