The variable circinate erythrokeratodermas comprise a rare group of genodermatoses having as their distinctive feature variable erythematous elements which take on bizarre shapes and are markedly influenced by environmental conditions. This is the report of a boy with features of this syndrome and a review of the literature. Erythrokeratoderma variabilis is the most frequently observed form, but other variants have been described which has led to confusion in nomenclature and classification. On present evidence a provisional grouping of these disorders seems indicated despite the fact that a consistent genetic pattern has not yet been observed. As more cases are recognized and pedigrees studied, the exact nosology of these unusual dermatoses may be clarified.