This paper shows a patient with clinical features of the TAR syndrome, who presents heterozigosis state for sickle cell anemia. The TAR syndrome was described by Shaw and Oliver in 1959, it is a rare genetic disease, occurring with an approximate prevalence of 1 case in 500.000 to 1 million births. Hall et al. described the current diagnostic criteria for TAR syndrome in 1969. These include bilateral absence of the radii in the presence of both thumbs and trombocytopenia. Other anomalies described included ulnar or humeral defects and the most severe cases phocomelia, lower limb involvement is variable, cadiac anomalies and cow’s milk intolerance (1,2) In this paper, we compare the findings of the literature review, discuss the ethiology and the importance of a diagnostic and treatment specially for trombocytopenia