Purpose: This study aimed to investigate the clinical features of macrocephaly at birth in Korea using ultrasonography. Methods: We retrospectively investigated the medical records of full-term birth neonates in Cheil General Hospital and Women’s Healthcare Center from January 2000 to June 2012. The following parameters were recorded and analyzed: gestational age, sex, birth weight, height, OFC (occipitofrontal circumference), physical examination, perinatal problems, and the result of ultrasonographly. Macrocephaly was diagnosed when the OFC was greater than 2 SD based on the 2007 Korean National Growth Charts. Results: There were 75 neonates with macrocephaly at birth (52 boys and 23 girls). The mean OFC was 38.1±0.49 cm. A comparison of the birth weight and height with the OFC showed that height correlates with OFC (r=0.35) but birth weight has no correlation with OFC (r=0.06). There was no remarkable finding in 56 cases (75%). Germinal matrix hemorrhage was found in 10 cases (13%). Enlarged CSF space was found in 5 cases (6.7%). In these patients, 3 cases had a megacisterna magna (4%); 1 case had a ventriculomegaly; 1 case had an enlarged interhemispheric space (6 mm). Choroid plexus cyst was seen in 1 case. Mineralizing vasculopathy in both basal ganglia with no evidence of congenital infection was found in 2 cases. Asymptomatic subarachnoid hemorrhage (SAH) was found in 1 case. Conclusion: Our results indicate that the macrocephaly at birth shows male dominance and has benign ultrasonographic findings.