Morgagni hernia is a type of congenital diaphragmatic hernia which constitutes 2–6% of congenital diaphragmatic hernia cases. Morquio syndrome, a type of lysosomal hydrolase deficiency, results in keratan sulphate accumulation and skeletal dystrophy. Other anomalies such as cardiac anomalies and trisomy 21 may accompany the Morquio syndrome; however Morgagni hernia has not been previously reported with this syndrome. A patient with Morquio syndrome that presented with bilateral Morgagni hernias is described. During surgery primary repair is preferred, however when there are large defects synthetic mesh grafts may also be used.