The diaphragm is formed by the fusion of several embryonic components which include the septum transversum, pleuroperitoneal membranes, esophageal mesentery, and body wall mesoderm . A congenital diaphragmatic hernia occurs when the pleuroperitoneal canal fails to close by 8th week of fetal gestation . The pathway of abnormal embryonic development leading to congenital diaphragmatic hernia is still incompletely understood. In the embryonic development period, fusion defects of the diaphragm can occur, resulting in postero-lateral defects (Bochdalek Hernia 70–75%), anterior-retrosternal defects (Morgagni Hernia 4%), and hiatal hernias and septum transversum defects (1%) . A BH is a congenital posterior lateral diaphragmatic defect resulting from a failure of the retroperitoneal canal membrane to fuse with the dorsal esophageal mesentery and the body wall , Vincent Alexander Bochdalek described the fusion defect of the postero-lateral foramina of the diaphragm in 1848 . The size of the hernia defect is variable and can range from 1 cm to almost complete agenesis of the hemidiaphragm. These hernias may contain stomach, spleen, colon, omentum, and small bowel. The fact that the left postero-lateral diaphragm closes after the right side may explain why the majority (~ 90%) of Bochdalek hernias occur on the left side. Right sided hernias are much rare and can involve the liver . The literature on Bochdalek hernia in association with hepatic heterotropia in infants is rather limited, with very few cases reported. The finding of hepatic heterotopia in association with a congenital diaphragmatic hernia is a rare occurrence and has been limited to few case report studies. Hepatic heterotopia can be classified into four main types: (1) a large accessory lobe of liver connected to liver by a stalk; (2) a small accessory lobe of liver connected to liver surface; (3) Ectopic liver without any connection to, forming a macroscopically identifiable nodule; (4) microscopic ectopic liver tissue . Types 2 and 3 have been reported with congenital diaphragmatic hernia, and our patient had type 2 hepatic heterotopia in the form of accessory lobe. The combination of CDH with an accessory liver is an extremely rare condition. Correction of congenital diaphragmatic hernia is possible in utero. However, liver involvement leads to significantly high mortality . To the best of our knowledge the association of accessory liver in combination with CDH in an infant is not the common occurrence. Aside from being fascinating incidental finding, the pediatric general surgeon should be aware of the clinical significance of this association. Firstly, associating this finding with cardiac anomaly as well as pericardial defect warrants careful examination and assessment of the pericardium through cardiac echocardiography, which is a routine workup of congenital diaphragmatic hernia . Secondly, knowledge of this association is helpful intra operatively to delineate the anatomy and facilitate the proper surgical repair of the diaphragmatic defect. Third, ectopic liver lobe may be complicated by secondary pathological condition such as torsion, infarction, and bleeding . While these complications were not reported in this case, it is essential for the surgeon to be aware of these Risks. Lastly, presence of hepatic heterotopia-like accessory liver lobe contribute to congenital diaphragmatic recurrence, thus stressing the importance of long term follow up for this particular anomaly is advised.