•Hypoplasia of the MV posterior leaflet is a rare congenital anomaly.•Differential diagnosis includes other anomalies of the mitral apparatus.•TTE and TEE are cornerstones for proper preoperative planning.•HPMVL tends to be incompatible with life.•Association of HPMVL with a BAV is extraordinarily rare.
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Velarde‐Acosta et al. (2024) studied this question.
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