Focal fatty change of the liver is a nodular lesion which is a rarely described and poorly characterized entity. The hepatic nodule measured 1.4cm at its maximum diameter, was subcapsular in location and occurred adjacent to the falciform ligament. Microscopically it was composed of hepatic tissue with a preserved lobular architecture. The central venous structures and portal tracts with their triads were regularly placed. The cytoplasm of almost all of the hepatocytes within the nodule was replaced by macrovesicular fat vacuoles with the nuclei displaced. Several large abnormal vessels were found at the margin of the nodule. The nodule was discovered incidentally on postmortem examination of a female infant who proved, at autopsy, to have multiple cardiac anomalies and bronchopneumonia. The possible inadequate local tissue perfusion due to abnormal intrahepatic vessels at this particular location could be augmented by multiple cardiac anomalies culminating in focal ischemia and focal fatty change. When encountered in surgery or on gross examination, it could be confused with other space occupying lesions such as liver cell adenoma, abscess and metastatic lesions.