Background:
Congenital anterior urethrocutaneous fistula is an exceptionally rare genitourinary anomaly that presents unique diagnostic and therapeutic challenges. This condition may occur as an isolated anomaly or in association with other abnormalities such as hypospadias and chordee, anorectal malformations, hypospadias, which can complicate clinical evaluation and management.
Objective:
To describe a congenital variant of chordee associated with an anterior urethrocutaneous fistula, review its etiology, and discuss current surgical management strategies.

Study Design:
Case report and comprehensive literature review.
Setting and Duration:
Department of Urology, Jinnah Postgraduate Medical Center, Karachi, Pakistan; January 2022 to January 2023.
Methods:
A detailed literature review was conducted to compile data on the etiology and surgical approaches for managing congenital anterior urethrocutaneous fistula, with a particular emphasis on cases involving chordee. We report a case involving a 14-year-old boy diagnosed with Congenital anterior urethral fistula associated with chordee. The diagnostic pathway, surgical intervention, and postoperative course are described.

Results:
Worldwide, only 67 cases of congenital anterior urethrocutaneous fistula have been reported. This anomaly may present as an isolated condition or alongside other abnormalities, including hypospadias and chordee. In our case, the presence of chordee did not hinder the successful correction of the fistula. The patient underwent urethroplasty with excellent postoperative outcomes, and follow-up in the outpatient clinic confirmed normal urinary function and a satisfactory cosmetic result.

We reported a case of urethrocutaneous fistula with chordee. The diagnostic pathway, surgical intervention, and postoperative course is described.

Conclusion:
Congenital anterior urethrocutaneous fistula, though rare, can be effectively managed surgically, even when associated with chordee. Surgical approach is indivisualized and is based on a number of features like site of fistula, patency of distal urethra and associated anomalies. Early recognition and tailored surgical intervention are paramount for optimal outcomes. Further studies are needed to enhance our understanding of its etiology and to refine management strategies.