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Living with Solitary median maxillary central incisor syndrome

A rare condition in which a child has only one upper front tooth, sometimes with other differences of the head and face.

Understanding Solitary median maxillary central incisor syndrome

Solitary median maxillary central incisor syndrome is a rare condition in which there is a single upper front tooth instead of two.

It affects both the baby tooth and the permanent tooth. Other features can include eyes set close together, a small head, short stature, a blocked back nasal passage and learning disability. Some people have a more serious brain difference called holoprosencephaly.

Signs & symptoms

Symptoms vary from person to person. GARD lists these among the most common:

  • Only one upper front tooth
  • Eyes set close together
  • Small head (microcephaly)
  • Short stature
  • Blocked back of the nasal passage
  • Learning disability

Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.

Diagnosis

  1. Talk with your doctor

    Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.

  2. See a specialist

    Your doctor may refer you to a specialist who knows rare conditions.

  3. Ask which tests can help

    Your care team will decide which exams and tests can confirm the diagnosis.

Care & treatment

Care team
A doctor familiar with rare conditions, plus specialists as needed
Expert care
Ask about multidisciplinary care centers for rare conditions
Treatment
GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
Research
Clinical trials and observational studies may be options. See the research section below.

General information, not medical advice. Your care team makes the decisions.

Everyday life

Living with a rare condition is easier with support.

  • Connect with Families for HoPE
  • Keep notes on symptoms, changes and questions for appointments
  • Ask about patient registries that help research
  • Look into financial aid and travel resources for care visits
Bring these questions to your next doctor visit
Can genetic testing help find the cause?
Should a brain scan or hormone tests be done?
Which dental specialists should we see?

Quick facts

InheritanceAutosomal Dominant

GARD doesn't list a gene. It is inherited in an autosomal dominant pattern, meaning one changed copy of a gene is enough.

Also known as: fused incisors; only one upper front tooth; single central incisor; single central incisor syndrome; single central maxillary incisor; single central upper incisor

Patient organizations

Source: NIH GARD · Solitary median maxillary central incisor syndromeGARD ID 4877 · GARD page updated September 2026 · Plain-language summary by Know Rare; editorial review pending. General information, not medical advice.

You are more than your diagnosis

Perspectives from people navigating life with Solitary median maxillary central incisor syndrome.

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