Mesenchymal hamartoma of the liver is a benign tumor resulting from abnormal, intra-uterine development of bile ducts and has a delayed clinical manifestation, thus this lesion appears to be related to the processes of maturation. It is potentially pre-malignant lesion presenting as a solid and/or cystic neoplasm. Symptoms, laboratory results and radiographic imaging are nonspecific and inconclusive, so surgical excision of the whole lesion is the imperative for the definitive diagnosis.

Recognition and diagnosis of the syndrome is of the exceptional importance. Proper and timely diagnosis should contribute to good outcome of the pregnancy and delivery.