BackgroundPeter’s anomaly is a rare congenital anterior segment dysgenesis with poor visual results. This case report describes a case of bilateral Type II Peter’s anomaly with notable clinical and histopathological features.Case presentationA 7-year-old boy was admitted to our center with complaints of bilateral central opacification, photophobia and severe reduced vision since birth. He underwent phacoemulsification, intraocular lens (IOL) implantation and anterior vitrectomy on the right eye in another medical institution two years ago. Slit lamp examination revealed bilateral central corneal opacity, few strands of peripheral iris, irregular pupils and cloudy lens with central adhesion to posterior corneal surface in the left eye. Additionally, a history of premature birth and mental retardation was also noted. The patient was diagnosed with Peter’s anomaly in the left eye, pseudophakia in the right eye and bilateral amblyopia. Similar surgery to the right one was performed on the left eye. A vesicle-like structure was found in the anterior chamber intraoperatively, which was composed mainly of immature lens and some corneal stroma as revealed by postoperative histopathological examinations.ConclusionsThe exact mechanism of Peter’s anomaly is not completely understood, however, the notable histopathological features of tissue obtained from the present case may provide evidence to the hypothesis of developmental anomalies.