Purpose. Bardet Biedal Syndrome (BBS) is a multiorgan syndrome affecting mainly the eyes, extremities, gonads __ampersandsign renal system etc. Method. Case report. Results The patient had common features of BBS like retinitis pigmentosa, mental __ampersandsign growth retardation, hypogenitalism, syndactyly and polydactyly along with atypical features like microtia, hemimandibular hypoplesia __ampersandsign pigeon shaped chest which are not reported in the literatures as for our best knowledge. Conclusions. Hemimandibular hypoplesia, microtia (small ear) __ampersandsign pigeon chest should also be considered as an association of Bardet Biedal Syndrome ( BBS).