In phenotypic females, absence of both ovaries usually is associated with an abnormal karyotype and a syndrome of gonadal dysgenesis. In such cases, bilateral streak gonads, or a unilateral streak gonad and a contralateral intra-abdominal testis are usually found.

Rarely, one ovary may be absent in an otherwise normal woman, usually representing an incidental finding at operation (Fig. 6.1). The differential diagnosis includes (1) ectopic ovary, which may lie at the level of the liver, close to the kidney, within the omentum, or within an inguinal hernia, and (2) adnexal torsion with atrophy or autoamputation.