Oculofaciocardiodental syndrome is a rare X-linked dominant genetic disorder.

Teeth with large roots (radiculomegaly), heart defects and small eyes (microphthalmia) are the characteristic triad found in this syndrome.

Typical features of the condition include:[citation needed] This condition is caused by lesions in the BCOR gene located on the short arm of the X chromosome (Xp11.4).

[citation needed] A genetically related disorder is Lenz microphthalmia syndrome.

The first features of this syndrome noted were the abnormal teeth, which were described by Hayward in 1980.