Thrombosis of umbilical cord vessels is associated with a high perinatal mortality. Most cases are venous thrombi, arterial thrombi are uncommon. To clarify clinical and pathological features of this entity, we reviewed 11 cases with umbilical artery thrombosis. The gestational ages were 33-40 (mean 36.8) weeks. Three cases (38%) were associated with severe intrauterine growth retardation and two cases (25%) led to intrauterine fetal deaths. All cases had completely occlusive thrombi of one umbilical artery. The arteries with thrombosis demonstrated partial necrosis of vascular wall. The pathogenesis is unclear, but nine cases (82%) had cord abnormalities (long cord, peripheral cord insertion, short cord with twist, funisitis). This paper is the first report of clinical and pathological features of umbilical artery thrombosis. The results of this study confirm the clinical importance and unique histological findings of umbilical artery thrombosis. The recognition of umbilical artery thrombosis is necessary to establish the diagnosis and treatment of this condition.