Objective To investigate the effectiveness of longitudinal preputial pedicled flap urethroplasty for chordee of Donnahoo IV type. Methods Between June 1994 and October 2011, 30 patients with chordee (Donnahoo type IV) underwent longitudinal preputial pedicled flap urethroplasty. The patients’ age ranged from 2 to 16 years (mean, 5.8 years). The morphology of the balanus-navicular fossa-external urethral orifice ranged normal; the penis length was 2.5-6.8 cm (mean, 4.3 cm); the penis bending angle was 35-70° (mean, 40.1°). Primary and secondary operation was 27 cases and 3 cases, respectively. The size of flap ranged from 1.5 cm × 1.3 cm to 4.0 cm × 2.0 cm. Results After correction, the penis length was 3.0-8.5 cm (mean, 6.6 cm); the penis bending angle was 0-10° (mean, 1.2°). All patients were followed up 6 months to 12 years (mean, 33 months). No recurrence, stabbing pain of the balanus, or foreign body sensation occurred during follow-up. Of them, 4 patients (13.33%) had urinary fistular, they had satisfactory results after the second operation; 2 patients (6.67%) had urethral stricture 1 month after operation, they also had satisfactory results after arethral dilatation. The other patients showed no scattering urinary flow and good direction without complication. Six patients had satisfactory sexual function after puberty without erection disorder, pain, or dyspareunia. Conclusion Longitudinal preputial pedicled flap urethroplasty can achieve maximum utilization of prepuce and aesthetic and functional improvement with less complication, so it is a relatively ideal mean for treating chordee of Donnahoo type IV.