Although McFarren et al have reported the case of DBA patient with CDH associated with mutations of RPS19 and RPS24 genes, the hernia was of a right-sided Morgagni type.[12] Therefore, to the best of our knowledge, this is the reported case of Bochdalek hernia in a DBA patient with RPS19 mutation. The gene discussed is RPS19; the disease is Posterolateral diaphragmatic hernia.