PURPOSE: We present a clinicopathologic case report of sclerocornea with the intent of showing the usefulness of ultrasound biomicroscopy (UBM) and the correlation of histopathologic findings. METHODS: An infant with congenital bilateral sclerocornea was seen for an evaluation under anesthesia. Dense opacification of both corneas prevented adequate examination of both anterior and posterior segments of the eye. UBM was performed preoperatively with subsequent corneal transplantation of the left eye. The corneal button was submitted for histopathologic examination. RESULTS: Preoperative UBM proved helpful in assessing the status of the cornea as well as the anterior chamber and its structures. Findings on histopathologic examination correlated well with the results on UBM and confirmed the diagnosis of sclerocornea. CONCLUSION: We recommend the use of UBM in assessing opacified corneas to assist in obtaining a diagnosis, to highlight potential associated structural anomalies, and to help guide decisions regarding surgical management.