Background and Purpose: Gender differences in stroke outcome have not been fully assessed in young patients. Methods: We conducted an observational study of consecutive young ischemic stroke patients (≤50 years of age) admitted to a stroke unit (January 1999 to December 2009). Basal data, subtype of ischemic stroke, stroke severity [Canadian Neurological Scale (CNS)], length of hospital stay, inhospital complications, mortality and functional outcome at discharge [modified Rankin Scale (mRS) score] were analyzed. For stroke severity and outcome analyses, 2 age groups were established: 15–30 (very young group) and 31–50 years old (middle-aged young group). Results: A total of 310 patients were enrolled; 128 females and 182 males. The mean age was similar in women and men (41.07 ± 8.6 vs. 42.12 ± 8.2, NS). Migraine was more frequent in women, whereas arterial hypertension, hyperlipidemia, alcohol abuse, current smoking and atherothrombotic infarction were more frequent in men (p < 0.05). Females presented greater stroke severity than men [median CNS (IQR) = 8 (3.5) vs. 9 (2.5), p = 0.014] except in the very young group [median CNS (IQR) = 9 (1.8) vs. 8 (5), p = 0.022]. Female sex was a predictor of unfavorable outcomes (mRS >2) at discharge in the total sample (OR = 3.33; 95% CI = 1.41–7.84) and in the middle-aged young group (OR = 2.62; 95% CI = 1.05–6.53), adjusted by baseline data, stroke subtype, inhospital complications, length of stay and stroke severity. Conclusions: Female gender is associated with worse outcomes in adult ischemic stroke patients up to 50 years old. However, this effect is not observed in younger patients (15–30 years).