The Triple A syndrome is a rare condition comprising achalasia, alacrima and adrenocorticotrophic hormone (ACTH) insensitivity. A 12 year old Chinese girl with a variant of this syndrome (achalasia and alacrima), presenting with failure to thrive, is reported. Typical appearances of achalasia on barium swallow subsequently led to the correct diagnosis. The radiologist may be the first to recognize this syndrome and hence help anticipate its potentially life-threatening sequelae.