Atypical myopathy is an acute intoxication induced by the ingestion of hypoglycin A, a toxin contained in the seeds and seedlings of certain trees of the genus Acer (particularly Acer pseudoplatanus [sycamore] in Europe), which results in disruption of the energy metabolism. The clinical signs arise from energy depletion in the type I fibres of the postural and respiratory muscles, and lead to a specific biochemical profile that may be used to confirm a diagnosis. Since there is no cure for the condition, the emphasis is on prevention, which requires knowledge of the conditions necessary for the causative agent to exert its toxicity. This article gives an overview of atypical myopathy, discussing the mechanism involved, its aetiology and the clinical signs and management (therapeutic and prevention).