&NA;Articles published between October 1991 and September 1992 on the ocular manifestations of metabolic diseases are reviewed. Articles of special interest include a study that shows that compliance to a low‐arginine diet in affected siblings with gyrate atrophy of the choroid and retina dramatically slows the progression of the chorioretinal dystrophy. The efficacy of cysteamine topical eye drops in the treatment of corneal crystals in patients with nephropathic cystinosis was described in a case report from Germany confirming the previous report of a clinical trial. The ophthalmic findings in 33 patients with classic galactosemia were reviewed. Sequential electroretinograms in two siblings with Batten's disease are described and help confirm that the primary lesion is in the inner retinal layers. And finally, clinical and histopathological studies of two families with lattice corneal dystrophy and type IV amyloidotic polyneuropathy (Meretoja syndrome) are presented.Current Opinion in Ophthalmology 1993,4;VI:83‐88