Two sisters and two unrelated patients with an ichthyosiform dermatosis resembling nonbullous ichthyosiform erythroderma were found to have lipid accumulations in the granulocytes of the peripheral blood, in the granulocyte precursors in the bone marrow, and in the liver. In each case, the blood anomaly was readily detected on examining a peripheral smear. One patient showed many of the features seen in Refsum syndrome. This association of findings in four patients may constitute another neurocutaneous lipidosis.