U, RamachandranT, MallaKS, Joshi2025-07-232025-07-232006https://hdl.handle.net/20.500.14572/563Ramachandran U 1, Malla T2 , Joshi KS 3 1Department of Paediatrics, Manipal Teaching Hospital, Pokhara, NepalMeckel- Gruber syndrome is a rare lethal, autosomal disorder. It has been linked to chromosome 17. It consists of a triad of occipital meningoencephalocoele, large polycystic kidneys and post-axial polydactyly. Death is mainly due to pulmonary hypoplasia. We report this rare case which presented with many associated defects. Key words: - MKS syndrome, meningoencephalocoeleen-USArticle