BACKGROUND: Results are conflicting as to whether sex has an impact on the outcome of intracerebral hemorrhage (ICH), especially when etiologies differ. In this study, we investigated whether sex differences exist in patients with vascular abnormality-related ICH. METHODS: Patients (age >= 18 years) diagnosed with ICH within 7 days of symptom onset were admitted consecutively between January 2012 and February 2014 from 50 hospitals across mainland China. Vascular abnormality related to ICH included aneurysm, arteriovenous malformation, moyamoya disease, and cavernous malformation. The outcomes were death and death/disability at 3 months. Disability was defined as modified Rankin Scale score >2. Multivariable logistic regression was used to estimate the association between sex and outcome. RESULTS: Women accounted for 41.9% (170) of the 406 patients, and they tended to be older than the men (women: 43.5 +/- 19.3 years; men: 40.0 +/- 17.7 years; P = 0.056). The proportions of ICH-related vascular abnormalities were as follows: aneurysm, 32% (130/406); arteriovenous malformation, 50.3% (204/406); moyamoya disease, 11.3% (46/406); and cavernous malformation, 6.4% (26/406). After we adjusted for age, National Institute of Health Stroke Scale, Glasgow Coma Scale score, location of hemorrhage, and surgery, female sex remained an independent predictor of death/disability at 3 months (odd ratio 2.49, 95% confidence interval 1.31-4.75), but not for death alone (odd ratio 1.45, 95% confidence interval 0.58-3.61). CONCLUSIONS: In our study, female sex was an independent risk factor for poor outcomes in patients with vascular abnormality-related ICH. The factors contributing to this sex difference should be investigated in the future.