Objective To investigate the local ization and expression characteristics of androgen receptor (AR) in genital tissue of patients with congenital hypospadias and simple chordee. Methods Between August 2005 and Janury 2007, dorsal prepuce, ventral perimeatal skin, and urethral plate were harvested from 25 patients with congenital hypospadias (aged from 1 year and 11 months to 19 years with an average of 3 years and 7 months) and 4 patients with simple chordee (aged from 3 years and 6 months to 16 years with an average of 7 years and 1 month). Prepuce by circumcision from 18 patients was used as control. The expression intensity and distribution of AR were assessed with mmunohistochemistry. Results AR was expressed in prepuce tissues from congentital hypospadias, simple chordee, and control. The AR positive cell rates were 62.94% ± 5.40% and 62.87% ± 5.33% in dorsal and ventral prepuce of control patients respectively, and were 59.00% ± 3.75%, 58.46% ± 4.14%, and 52.30% ± 3.53% in dorsal prepuce, ventral perimeatal skin, and urethral plate of patients with congenital hypospadias respectively. AR positive cell rate was significantly lower in patients with congenital hypospadias than in control patients (P lt; 0.05), and in urethral plate than in dorsal prepuce and ventral perimeatal skin of patients with congenital hypospadias (P lt; 0.05), and no significant difference was detected between dorsal and ventral specimens (P gt; 0.05). Stratified analysis showed a similar expression mode in severe hypospadias group and severe chordee group (P lt; 0.05). In mild to moderate hypospadias group and mild to moderate chordee group, no significant difference was shown when dorsal and ventral skin specimens were compared to that in normal control (P gt; 0.05), with AR expression diminished in urethral plate (P lt; 0.05), and AR decrease was relative to severity of chordee (P lt; 0.05). The AR positive cell rates were 59.69% ± 2.73%, 55.71% ± 1.67%,and 51.92% ± 1.87% in dorsal, ventral skin, and urethral late of patients with chordee respectively. Reducing tendency of AR expression was observed. Conclusion AR expression decreases in penile skin of patients with congenital hypospadias and simple chordee, especially in urethral plate.