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While \u201cclassic\u201d eye findings, including the spectrum of midline anomalies ranging from cyclopia to hypotelorism, as well as chorioretinal coloboma and microphthalmia, have been frequently described in patients with HPE, other subtle eye anomalies may also occur. In our study we prospectively analyzed a small cohort of 10 patients in whom we identified mutations in <jats:italic>SHH<\/jats:italic>, <jats:italic>SIX3<\/jats:italic>, <jats:italic>ZIC2<\/jats:italic>, or <jats:italic>FGF8<\/jats:italic>, the latter of which is a very recently described HPE\u2010associated gene. We found that 9 of 10 patients had at least two ophthalmologic anomalies, including refractive errors, microcornea, microphthalmia, blepharoptosis, exotropia, and uveal coloboma. These findings contribute to the understanding of the phenotypic variability of the HPE spectrum, and highlight findings in one medically important but often incompletely investigated system. Published 2011. 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