Objectives: Though mitochondrial disorders due to a respiratory chain defect may manifest with haematological abnormalities such as anaemia, neutropenia, or thrombocytopenia, recurrent steroid-responsive eosinophilia has not been reported as a manifestation of a mitochondrial disorder.Clinical presentation and intervention: In a polymorbid 65yo female recurrent erythema, asthma, chronic bronchitis, gastritis with eosinophilic granuloma, recurrent episodes hypereosinophilia, and polyarthralgia suggested Churg-Strauss syndrome, which was excluded upon absence of eosinophilic vasculitis on any of the biopsies. However, corticosteroids were effective for hypereosinophilia. In addition to the hypereosinophilic syndrome a mitochondrial disorder with multi-system affection was diagnosed upon short stature, basal ganglia calcification, pituitary adenoma, hypoacusis, tinnitus, atrial fibrillation, diffuse weakness of all four limbs, diffuse wasting, tetraspasticity, muscle aching, restless-leg syndrome, axonal neuropathy, polyarthralgia, osteoporosis, arterial hypertension, occasional thrombocytopenia, hypercholesterolaemia, hyponatriaemia, elevated liver function parameters, and a muscle biopsy indicative of a mitochondrial disorder. Hypereosinophilia was interpreted as a manifestation of the mitochondrial disorder after exclusion of various differentials.Conclusion: Mitochondrial disease may go along with marked eosinophilia mimicking Churg-Strauss syndrome. Steroids may be useful to resolve episodic eosinophilia but may be ineffective and contraindicated for mitochondrial myopathy.