Spontaneous serous detachment of the choroid and ciliary body, together with bullous serous detachment of the retina (uveal effusion syndrome) is a rare but well-defined syndrome frequently associated with nanophthalmos. CASE REPORTS: Based on three cases whose one familial, clinical and ultrastructural characteristics of the syndrome are reviewed and the role of ultrasonic biomicroscopy (UBM) presented. CONCLUSION: The severe and potentially blinding complications encountered after any surgery on these eyes make the recognition of the classical clinical signs and symptoms of the syndrome particularly important. In this context, UBM represents an interesting new tool in terms of diagnosis and pathophysiology understanding.