PURPOSE: Although urachal anomalies are rarely observed clinically, they often give rise to a number of problems, such as infection and late malignant changes. Because of variable clinical presentations, uniform guidlines for evaluation and treatment are lacking. The authors discuss the problems involved in both the diagnosis and the treatment of these anomalies. METHODS: We retrospectively analyzed 15 patients (9 males and 6 females) who had undergone surgery for urachal anomalies from July 1995 to December 1999. RESULTS: The age distribution ranged from 14 days to 38 years old, and the male to female ratio was 1.5:1. The 4 variants of urachal anomalies included a urachal sinus in 7 patients (47%), a urachal cyst in 4 (27%), a patent urachus in 3 (20%), and a bladder diverticulum in 1 (6%). The presenting complaint was periumbilical discharge in 6 patients, fever in 5, umbilical granuloma in 4, low abdominal mass in 3, and low abdominal pain in 1. Eight combined anomalies were seen in 7 patients; 2 umbilical hernias, 1 inguinal hernia, 1 hydrocele, 1 urachal vessel anomaly, 1 vesicoureteral reflux, 1 hydronephrosis, 1 Hirschsprung's disease, and 1 hypertrophic pyloric stenosis. Ultrasound examination disclosed a cyst or a sinus in 5 patients, and CT was performed in 1 case. Excision was performed in all patients, and there was no postoperative complication or recurrence. CONCLUSION: Urachal anomalies most frequently present in infancy or childhood, and the initial presentation is umbilical discharge with infection. Furthermore, the large number of associated genitourinary and gastrointestinal anomalies suggests that a complete work-up for these conditions should be performed. Definitive surgical excision appears to be appropriate for most patients.