Objective To investigate the clinical features of Takayasu arteritis patients with different genders and ages. Methods The medical charts of 397 patients diagnosed with Takayasu arteritis in Changhai Hospital, Naval Medical University (Second Military Medical University) between Jan. 2001 and Dec. 2017 were retrospectively reviewed. The clinical manifestations, complications, laboratory parameters and imaging findings were compared between male and female patients, and between the groups subdivided by the ages of onset (≤ 40 years vs >40 years). Results There were 64 males and 333 females, with a male to female ratio of 1:5.2. The age of disease onset was 2-67 years. Seventeen (26.6%) male patients and 53 (15.9%) female patients had disease onset after 40 (χ²=4.190, P=0.041). Compared with female patients, male patients had their onset more frequently with hypertension (31.2%[20/64] vs 16.5%[55/333], χ²=7.605, P<0.01) and less frequently with dizziness, headache and syncope (28.1%[18/64] vs 46.5%[155/333], χ²=7.409, P<0.01). During the course of disease, hypertension was more prevalent (70.3%[45/64] vs 56.8%[189/333], χ²=4.076, P<0.05) and diminished or absent pulse was less common (51.6%[33/64] vs 76.0%[253/333], χ²=15.885, P<0.01) in male patients. Male patients with onset age ≤ 40 years (versus >40 years) had a higher incidence of hypertension (78.7%[37/47] vs 47.1%[8/17], χ²=5.997, P<0.05) and a lower incidence of diminished or absent pulse (40.4%[19/47] vs 82.4%[14/17], χ²=8.787, P<0.01). The complication rates of aneurysm and aortic regurgitation were significantly higher in male patients versus female patients (39.1%[25/64] vs 17.4%[58/333], 23.4%[15/64] vs 10.8%[36/333]; χ²=15.210, 7.644; both P<0.01). The prevalence rates of elevated erythrocyte sedimentation rate or C-reactive protein were similar between different gender and onset age groups (all P>0.05). Angiographically, male patients had more renal artery involvement and type Ⅳ (54.7%[35/64] vs 32.7%[109/333], 32.8%[21/64] vs 18.9%[63/333]; χ²=11.194, 6.212; both P<0.05) and less carotid artery and subclavian artery lesions and type Ⅰ (37.5%[24/64] vs 64.3%[214/333], 37.5%[24/64] vs 56.5%[188/333], 21.9%[14/64] vs 42.3%[141/333]; χ²=16.016, 8.416, 9.449; all P<0.01) compared with female patients. Male patients with onset age ≤ 40 years (versus >40 years) had a higher proportion of type Ⅳ (44.7%[21/47] vs 0[0/17], χ²=11.305, P<0.01) and lower proportions of carotid artery and subclavian artery lesions and type Ⅰ (29.8%[14/47] vs 58.8%[10/17], 25.5%[12/47] vs 70.6%[12/17], 14.9%[7/47] vs 41.2%[7/17]; χ²=4.491, 10.814, 5.046; all P<0.05). Conclusion Male Takayasu arteritis patients have a later onset age compared with female patients. Hypertension and renal artery involvement (type Ⅳ) are commonly seen in male patients with onset age ≤ 40 years; female patients and male patients with onset age >40 years often have diminished or absent pulse and carotid artery and subclavian artery lesions (type Ⅰ). The complications such as hypertension, aneurysm and aortic regurgitation are more common in male patients compared with female patients.