Idiopathic infantile arterial calcification (IIAC) is a rare disease of unknown etiology, which is characterized by arterial calcification. A 29-year-old primigravida at 33 weeks’ gestation was referred for further evaluation for polyhydramniosis. An ultrasonographic examination revealed an intrauterine growth restricted fetus, pericardial effusion, increased renal cortical echogenicity with sparing of corticomedullary differentiation, and diffuse arterial calcifications involving the aorta, pulmonary artery, common iliac arteries, renal arteries, and common carotid arteries. At 35 weeks of gestation a cesarean section was performed because of fetal distress. A 1,900 g male infant was delivered. Postnatal examination confirmed the diagnosis of IIAC with dysmorphic features (clinodactily and low-set ears) and normal constitutional karyotype. The baby died when he was four months old in the newborn care unit. During routine obstetric ultrasonography, the combination of polyhydramniosis and intrauterine growth restriction may necessitate examination of the major vessels for presumptive a diagnosis of IIAC.