Introduction: Idiopathic antenatal calcification is a rare, generally lethal, condition with unclear etiology.
Materials and methods: Around 200 cases, most of them undergoing postnatal diagnosis, are reported in literature. The majority of the affected infants die before the age of 6 months, and very few have survived for more than 1 year. Five cases of spontaneous resolution of the disease are described.
Discussion: An autosomal recessive pattern of inheritance has been suggested for this condition, with some gene mutations which have been recently discovered. Therapy with bisphosphonates has been suggested, with conflicting evidence regarding the utility in the regression of the disease.
Conclusion: The main purpose of the present report is to provide the available knowledge on this subject through a systematic review of the literature. In addition, we describe two cases of antenatal idiopathic arterial calcification in which antenatal diagnosis was achieved.