Triple A syndrome is a rare, autosomal recessive disease. It is characterised by a triad of symptoms: alacrima, adrenocortical insufficiency and oesophageal achalasia. Atrophy of the optic nerve and a number of neurological disorders also occur in patients with this syndrome. Only about one hundred cases have been described in the literature since the first description by Allgrove in 1978. This paper presents a case report of siblings diagnosed with triple A syndrome. A 6-year-old girl developed adrenocortical insufficiency, axonal-demyelinating polyneuropathy and oesophageal achalasia, while her 3-year-old brother showed an abnormal adrenal reserve. Both children presented with the lack of tears during crying.