Allgrove or triple A syndrome (AS or AAA) is a rare autosomal recessive syndrome 
with variable phenotype due to mutations in AAAS gene which encodes a protein 
called ALADIN. Generally, it's characterized by of adrenal insufficiency in 
consequence of adrenocorticotropic hormone (ACTH) resistance, besides of 
achalasia, and alacrimia. Neurologic features are varied and have been the 
subject of several case reports and reviews. A few cases of Allgrove syndrome 
with motor neuron disease have been already described. A 25-year-old white man, 
at the age of four, presented slowly progressive distal amyotrophy and weakness, 
autonomic dysfunction, dysphagia and lack of tears. He suffered later of 
orthostatic hypotension and erectile dysfunction. He presented distal amytrophy 
in four limbs, tongue myofasiculations, alacrimia, hoarseness and dysphagia due 
to achalasia. The ENMG showed generalized denervation with normal conduction 
velocities. Genetic testing revealed 2 known pathogenic variants in the AAAS 
gene (c.938T>C and c.1144_1147delTCTG). Our case presented a distal spinal 
amyotrophy with slow evolution and symptoms and signs of AS with a mutation in 
AAAS gen. Some cases of motor neuron disease, as ours, may be due to AAS. Early 
diagnosis is extremely important for symptomatic treatment.