Background: Congenital unilateral renal agenesis occurs in 0.93–1.8 per 1000 autopsies, and is usually diagnosed on an incidental imaging examination. Genital anomalies occur in 37–60% of females and 12% of males
with congenital unilateral renal agenesis. Abnormalities in females include agenesis, duplication, rudimentary, unicornuate or bicornuate uterus, double or absent vagina, absent or hypoplastic ovary, absent fallopian tube,
abnormal external genitalia and others
Case Report: A 16-year-old female with congenital unilateral renal agenesis, ipsilateral ovarian agenesis and unicornuate uterus is presented. Abdominal and pelvic ultrasound revealed a hypertrophied right kidney measuring
13.0 cm, a normal right ovary, and absent left kidney and ovary. Computerized tomography of the abdomen and pelvis confirmed absence of the left kidney and ovary and revealed a unicornuate uterus with absent left horn.
Conclusions: Recognition of a congenital solitary kidney is important in order to monitor the affected individual for urinary infection, obstruction or calculi and warn the individual to avoid contact sports and similar activities
that might endanger the solitary kidney. Physicians should be aware of the association of congenital unilateral renal agenesis, ovarian agenesis and unicornuate uterus. Early detection of a congenital solitary kidney by
routine prenatal ultrasonography or incidental imaging should alert physicians to look for associated genital anomalies and avoid unnecessary procedures and surgery in patients presenting with abdominal and pelvic complaints.

Keywords: unilateral renal agenesis, ovarian agenesis, unicornuate uterus