The combination of placental mesenchymal dysplasia and hepatic mesenchymal hamartomas is an extremely rare finding. We present the case of a three-month-old female born at 35 weeks gestation with a history of placental mesenchymal dysplasia who presented with non-bilious, non-bloody emesis, and episodes of respiratory distress due to multiple enlarging abdominal cystic lesions. The patient’s presentation was unique due to both liver and adrenal solid and cystic lesions. After extensive imaging and multiple biopsies, expert interpretation of biopsy tissue revealed hepatic mesenchymal hamartoma within the liver and the adrenal gland. To our knowledge, this is one of the few documented cases of unresectable hepatic mesenchymal hamartomas with adrenal involvement successfully undergoing a whole liver transplant.