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BACKGROUND: The idiopathic uveal effusion syndrome is characterized by a spontaneous serous detachment of the choroid and ciliary body. Pathophysiology is related to a relative scleral impermeability to protein. A possible complication is the development of a serous retinal detachment. HISTORY AND SIGNS: We present a 65-year-old male, mild hyperopic patient with a peripheral circular choroidal tumour OD, and macular retinal folds OU. Visual acuity was OD 0.5 due to an additional cataract and OS 1.0. Ocular inflammation was not present. Extensive medical, serological and immunological investigations were normal. On MRI a thicker sclera compatible with the diagnosis was measured. THERAPY AND OUTCOME: Twelve months after the first presentation the patient has no complaints and the ocular findings are stable so that a therapy was not necessary up to now. CONCLUSIONS: Uveal effusion syndrome is a rare disease which belongs to the differential diagnosis of choroidal tumour with serous retinal detachment. The correct diagnosis is important as sclerectomy or sclerotomy may be an effective treatment.