LD2A.0

ICD-11 MMS 2026-01

Ovotesticular disorder of sex development

Description

Ovotesticular disorder of sex development, formerly called true hermaphroditism, is a rare cause of genital ambiguity characterised by the presence of ovarian and testicular tissue in an individual, leading to development of both male and female structures.

Index terms

  • Ovotesticular disorder of sex development
  • Ovotestis
  • True hermaphrodite
Show allHide
  • True hermaphroditism

Exclusions from above levels

  • 5A71 – pseudohermaphroditism: female, with adrenocortical disorder
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LD2A.1 – 46,XY gonadal dysgenesis
  • LD2A.2 – Testicular agenesis
  • LD2A.3 – 46,XY disorder of sex development due to a defect in testosterone metabolism
  • LD2A.4 – 46,XY disorder of sex development due to androgen resistance
  • LD2A.Y – Other specified malformative disorders of sex development
  • LD2A.Z – Malformative disorders of sex development, unspecified

Foundation entity

Ovotesticular disorder of sex development – http://id.who.int/icd/entity/989785304

Part of: LD2A: Malformative disorders of sex development › 20: Developmental anomalies