46,××男性综合征是一种罕见的性反转畸形,它是一种具有女性染色体组成而表型为男性的综合征。据 De Ia Chapelle 统计,其发生率约为二万分之一。自1964年首例发现至今,近三十年内,全世界仅有100余例报道。我科近来收治一例。病例报告患者男,35岁,未婚。因阴茎小,勃起时向腹侧弯而入院。患者出生后外生殖器无明显异常,被当做男孩抚 46, XX male syndrome is a rare sexual inversion deformity, it is a female with a chromosome composition and phenotype of men’s syndrome. According to De Ia Chapelle, the incidence is about one in 20,000. Since the first discovery in 1964, nearly 100 cases have been reported in the world in nearly three decades. I recently admitted to a case. Case report Male patient, 35 years old, unmarried. Due to small penis, erectile to the ventral bend and hospitalization. No abnormalities were found in the external genitalia after the patient was born, being treated as a boy