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Abstract Bardet‐Biedl Syndrome (BBS) is a rare autosomal recessive disorder with a wide spectrum of clinical manifestations like retinal dystrophy, obesity, polydactyly, mental retardation, hypogonadism, and renal dysfunction. We report a case of 14‐year‐old obese boy with poor scholastic performances, hypothyroidism, and poor vision diagnosed as BBS.