Ambiguous Genitalia C0266362
Ambiguous Genitalia refers to reproductive anatomy that does not clearly appear typical for male or female. It can result from various genetic, hormonal, or developmental factors. Care involves careful evaluation, family counseling, and sometimes surgical considerations.
Key facts at a glance
- ICD-10
- N39.0
- ICD-11
- GA00.0
- Body system
- genitourinary
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(genitourinary)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Ambiguous genitalia
(genitourinary)Hypospadias
(genitourinary)Clitoromegaly
(genitourinary)Gonadal dysgenesis
(genitourinary)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Genetic and endocrine workup
Comprehensive laboratory evaluation including karyotyping, hormone levels, and genetic testing to determine underlying etiology of ambiguous genitalia.
Multidisciplinary gender counseling
Expert psychological evaluation and family counseling to support gender assignment decisions, respecting cultural, medical, and family considerations.
Additional treatment options
Gonadal biopsy and preservation
Surgical evaluation of gonadal tissue to assess malignancy risk and consider fertility preservation options when appropriate.
surgicalLong-term endocrine follow-up
Lifelong monitoring of hormonal function, growth, and development by endocrinology specialists familiar with intersex conditions.
monitoringGenitoplasty when indicated
Surgical intervention to align external genitalia with assigned gender, performed after careful evaluation and family counseling when medically indicated.
surgicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Ambiguous Genitalia C0266362?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Lee PA, Nordenström A, Houk CP, et al. Global disorders of sex development update on human genetics and management. Endocr Rev. 2016.
Ahmed SF, Achermann JC, Arlt W, et al. UK guidance on the initial evaluation and long-term management of DSD. Arch Dis Child. 2016.
Meyer-Bahlburg HF, Baratz Dalke K, Berenbaum SA, et al. Gender development in 46,XY children with DSD: a prospective longitudinal study. J Clin Endocrinol Metab. 2020.
Houk CP, Lee PA. Consensus statement on terminology and management of disorders of sex development. Int J Pediatr Endocrinol. 2018.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy