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A new variant of hereditary thrombocytopenia combined with giant platelets and inclusion bodies in the leukocytes different from those found in the May Hegglin anomaly and from Döhle bodies is presented. Investigations of platelet function, platelet nucleotides and clotting studies revealed normal results. Beside their size and spherical shape platelets did not show morphologic abnormalities. The inclusion bodies were similar to those found in the Fechtner syndrome, a variant of Alport's syndrome. However, the feature of Alport's syndrome, including high frequency deafness, congenital cataracts, and chronic interstitial nephritis are absent in the family pedigree. Thus the Sebastian platelet syndrome represents a unique syndrome with giant platelets and inclusion bodies in the leukocytes.