In 1946, Potter descrived a series of 20 cases of infants in whom bilateral absence of the kidneys was associated with hypoplasia of the lungs and a characteristic face. This combination of abnormal findings has been known as Potter's syndrome after the first describer, E.L. Potter. Since these anomalies are incompatible with life, the diagnosis of this syndrome was seldom made clinically only to be found incidentally at autopsy table. this may be the reason why this anomaly was not reported in this country until very recently by us Chi and Park, in spite of over 160 cases reported in the literature. This case would be the second case of Potter's syndrome in Korean literature.