Introduction: Patent urachus comprise among the spectrum of congenital urachal anomalies. Literature reports just over 100 cases in the neonatal period as documented in the literature so far.

Materials & methods: We report a case of a 7 day old baby, presenting with urine discharge through umbilicus. Ultrasound of the abdomen showed absent right kidney with normal left kidney. MCUG finding showed fistulous track between umbilicus and urinary bladder with features suggestive of patent urachus.

Result: Open surgical excision of the urachus was done in our case with no complication. Histopathological findings were consistent with urachal epithelium.

Conclusion: Urachal anomalies may present in various forms. Symptomatic urachal anomalies should be surgically excised. Treatment is complete excision of the urachal tract along with a cuff of bladder, which can be done either by open surgery or laparoscopic–endoscopic approach