Rieger’s anomaly is a rare congenital ocular defect with autosomal dominant inheritance, characterised by dysgenesis of the anterior segment. Ocular features of typical Reiger’s anomaly include a prominent anteriorly displaced Schwalbe's line (posterior embryotoxon), iris stromal hypoplasia, corectopia, and glaucoma. An atypical presentation of Rieger’s anomaly is described in the current case report. A 26-year-old female presented with diminution of vision in right eye and mild photophobia in both eyes, since childhood. On examination, it was recognised as an atypical isolated case of Rieger’s anomaly with several classical features including segmental full thickness iris defect and ectropion uveae. This was associated with total cataract in right eye and persistent pupillary membrane in left eye, without posterior embryotoxon and glaucoma. There were no other associated ocular or systemic anomalies. Patient was operated for cataract surgery in right eye under guarded visual prognosis. The postoperative visual acuity was 3/60, signifying pre-existing amblyopia. The patient was kept on close follow-up for the development of glaucoma. This is a rare case of Anterior Segment Dysgenesis (ASD) manifesting as atypical Rieger’s anomaly without posterior embryotoxon and any systemic association, signifying the fact that posterior embryotoxon is not an essential diagnostic criterion.