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Diagnsis of TAR syndrome usually are made at birth because of the characteristic physical appearace combined with thocmbocytopenia. The Two essential features of TAR syndrome are hypomegakaryocytic thrombocytopenia and bilateral radial aplasia. The rest of the phenotype varies widely and can manifest with abnormalities involving skeletal, skin, gasterointestinal and cardiac systems. Considering to limited cases of syndrome and variability of phenotypic abnormalities exess two essential features, all cases of this syndrome are reportable. We reported a patient with TAR syndrome with any related malformations.