Objective: To investigate the clinical phenotype, psychological effects, and coping strategies of patients with Mayer-Rokitansky-Kuster- Hauser (MRKH) syndrome in China. Materials and Methods: The authors enrolled 46 patients with MRKH syndrome and collected the data on clinical manifestations by physical examination, psychological impact, and mental functions by self-administered questionnaires, and coping strategies through the medical questionnaire (MCMQ). Results: Among 46 patients with MRKH, 34 (73.91%) were classified as type I and 12 (26.09%) as type II; patients in the type II group had renal abnormalities and/or skeletal deformities, 60.86% (28/46) of the patients had feeling of anxiety, fear, and upset, 52.17% (24/46) were troubled by the disease, and 56.52% (26/46) were frustrated and had depression. However, 67.39% (31/46) of the patients were satisfied with their social status and family relations as well as perception of surrounding people. The results from the survey on the patients' strategies of coping with the disease showed that the mean scores (and SDs) of the facing, avoidance, and yielding dimensions were 18.30 ± 3.74, 17.05 ± 2.52, and 11.30 ± 4.37, respectively. The psychological function scores were significantly correlated with the scores of yielding dimension (r = 0.613, p = 0.004). Conclusion: The health impact of MRKH syndrome includes not only its physiological function, but also social and psychological aspects. The Chinese patients with MRKH syndrome often use yielding or avoidance strategies to cope with the disease. A comprehensive care and supporting program including medical, psychological, and social/educational components should be developed for MRKH patients in China.