Thrombocytopenia and absent radii (TAR) syndrome is a rare disorder. It may be associated with for example, as cardiovascular, genitourinary, gastrointestinal, skeletal, neurological, ophthalmic, and facial anomalies. Esophageal atresia is also a rare anomaly associated with genitourinary, skeletal, and cardiovascular anomalies, among others. Here, the authors present a child with TAR syndrome and esophageal atresia with tracheoesophageal fistula. This association has been reported in only one other case in literature.