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SUMMARY We report a family in which the father and all three children had symptomless chronic renal failure and, in the case of the children, normocytic, normochromic anaemia. None had hypertension, proteinuria, or abnormality of urinary deposit. Renal biopsy specimens showed microcysts confined to the renal cortex; some cysts contained vestigial glomerular tufts.This family appears to represent the first known example of hereditary cortical microcystic disease. The distribution of the disease suggests dominant inheritance without sex linkage.Cysts of the renal cortex are common but are usually large, solitary, and of little clinical importance except in differential diagnosis.The various forms of polycystic disease are much less common. The familial types so far described have been either in the infantile nephrotic syndrome or part of a multisystem disorder. In this paper we describe a previously unrecognised form of the condition with no extrarenal abnormality and without the nephrotic syndrome. The father and all three children are affected. Family SInitially the three children were seen because of transient minor ailments by their family doctor, who found they were anaemic. Both parents and children felt well and even systematic questioning failed to elicit any symptoms. The children and father were pale, but the mother was of normal complexion. All were of normal stature except for the middle child, NS, whose height was only 1-25 m (below the third centile of age) and weight was 25-2 kg (third centile). Detailed examination of major systems revealed no abnormality. Blood pressures were normal as were optic fundi, and urine tests were negative for protein, blood, and glucose.The parents (JS and TS) came from the west of Ireland and their marriage was not consanguineous.Neither was aware of renal disease affecting parents