DOI: https://doi.org/10.36347/sasjm.2026.v12i09.029

Pages: 984-988

Background: Prune-Belly syndrome (PBS) is a rare congenital disorder defined by the triad of abdominal wall muscular deficiency, severe urinary tract anomalies, and bilateral cryptorchidism. Genital anomalies affecting the prostate and seminal vesicles are less well studied in adulthood, particularly regarding their impact on ejaculatory function and fertility. Case presentation: We report the case of a 20-year-old single male student, followed since childhood for complete PBS bilateral vesico-ureteral reimplantation on a psoas-hitch bladder for bilateral megaureter and bilateral orchidopexy at age 6, simple right nephrectomy for pyonephrosis in 2021, an annually exchanged left double-J stent, and clean intermittent self-catheterization. He presented with orgasmic anejaculation since the onset of sexual activity. Clinical examination was unremarkable (BMI 25.2 kg/m², normally positioned urethral meatus, scrotal testes of normal size and firm consistency, palpable vasa deferentia without abnormality, no varicocele or palpable mass). Transrectal ultrasound failed to identify any prostatic tissue. Pelvic MRI confirmed complete prostatic agenesis together with agenesis of the right seminal vesicle, with probable ectopic termination of the right vas deferens into the left seminal vesicle. Hormonal work-up (FSH 12.8 IU/L, LH 7.1 IU/L, testosterone 11.76 IU/L) showed no major abnormality. Discussion: The combination of prostatic and seminal vesicle agenesis with PBS is an exceptional, likely under-reported, finding that highlights the embryological relationship between urogenital sinus derivatives and Wolffian duct derivatives in this condition. The resulting anejaculation reflects an anatomical rather than a neurogenic or psychogenic mechanism, which has direct implications for fertility management, favoring surgical sperm retrieval over penile vibratory stimulation or electroejaculation. Conclusion: This case highlights the value of systematically assessing sexual functi