Prostatic utricles are commonly associated with hypospadias and differences of sex development (DSD) but are rarely large. We report a 6-month-old infant with 46,XX SRY-negative DSD and distal hypospadias, found to have a large pelvic cystic lesion measuring up to 6.7 cm. Serial imaging suggested a Müllerian duct remnant, while micturating cystourethrogram (MCUG) confirmed communication with the posterior urethra and a markedly enlarged prostatic utricle consistent with Ikoma grade III. The patient underwent cystoscopy-guided catheterization and laparoscopic excision. Complete removal was achieved with preservation of adjacent structures and an uncomplicated recovery. This case highlights the diagnostic value of MCUG and demonstrates that minimally invasive excision provides excellent visualization and enables safe excision in infancy.
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