Allgrove Syndrome (AS) is rare autosomal recessive disorder characterised by achalasia cardia, alacrimia and adrenal insufficiency, which is generally adrenocorticotropic hormone (ACTH) resistant, and neurological abnormalities. Mutations have been identified in the AAAS gene, located on chromosome 12q13 (type-2 keratin gene), that codes for the ALADIN protein. IVS14 and EVS9 are the most common mutations. Alacrimia is an early and pathognomic symptom, but achalasia (50 -100 %) and adrenal insufficiency (20 -54%) are the more common presenting features. Autonomic disturbances and other neurological symptoms (10- 23%) are rare. Patients may develop a variable combination of sensory-motor polyneuropathy amyotrophy, dysarthria, hyperreflexia, muscle weakness, dementia, abnormal autonomic function, erectile dysfunction (adult) and intellectual impairment. Diagnosis is generally made in the first decade of life when they present with dysphagia, vomiting and failure to thrive due to achalasia, hyperpigmentation of skin, shock due to adrenal insufficiency or seizures and coma due to severe hypoglycaemia. Typical dysmorphic faces including long thin face, long philthrum, narrow upper lip, down-turned mouth and sparse eyelashes may also be seen. Keratitis punctata is the most common complication of alacrimia. Patients with adrenal insufficiency are generally on a maintenance dose of a glucocorticoid like hydrocortisone. Most patients with achalasia require frequent pneumatic dilatations or surgical interventions like Heller’s myotomy.