While this kindred and the one described herein are in many respects similar, the diagnosis in the proband from the first kindred was made only on presentation of advanced thyroid carcinoma despite the presence of prominent corneal nerves noted some 15 years earlier.4 The corneal findings in the proband of our kindred led to identification of the syndrome and the decision to perform a prophylactic thyroidectomy. Because patients with MEN 2A do not exhibit the other classic phenotypic features of MEN 2B, it is essential that the clinical significance of prominent corneal nerves is recognized and acted on. Based on this case series, given that there were no other concerning features on history or examination of the index case, it is reasonable to consider genetic testing in all patients with prominent corneal nerves. However, as this study shows, not all individuals with this mutation will show evidence of prominent corneal nerves. To detect those with systemic manifestations of the MEN 2A syndrome, screening of family members is indicated regardless of the finding of normal corneas.