Studies were conducted on six new and four previously reported patients with the Sjögren-Larsson syndrome. Characteristically they showed generalized ichthyosis without significant lesions of the scalp, hair, or nails. Spastic paralysis, impairment of intelligence, and other neurologic changes were usually present. Other alterations of the eyes, teeth, and skin confirm the opinion that the condition is autosomal recessive in inheritance, with dermatological findings of significance.