We report a case of Bazex-Dupré-Christol (BDC) syndrome in a girl who died of prolymphocytic leukaemia at the age of 10 years. This is the first published case where the syndrome is associated with a blood disease. A review of the literature has enabled us to collect the various symptoms of BDC syndrome and to discuss the relationship of the syndrome with basal cell nevomatosis. We do not think that the blood disease was incidental. The child was 5 years' old in June 1972 when she was first seen in our out-patient clinic for severe hypotrichosis which had been present at birth and involved the eyelashes and eyebrows. Examination of the skin showed numerous milia on the face, a small lenticular polycystic lesion on the right ear, follicular atrophodermia on the back of the hands and hypotrichosis of the face. The right ear lesion was removed and found to look like Winer's cutaneous calcinosis. At light microscopy, many hairs showed like pili torti or, more rarely, like trichorrhexis nodosa. Despite the absence of basal cell carcinoma, the diagnosis of BDC syndrome was regarded as final. The patient died aged 10 years of prolymphocytic leukaemia revealed by pancytopenia. Combined chemotherapy with Rubidomycin, Oncovin and methotrexate had proved ineffective. Having collected from the literature 44 adequately documented cases summarized in table I, we describe the anatomico-clinical manifestations of Bazex-Dupré-Christol syndrome. Follicular atrophodermia is present in 85 p. 100 of the cases, but its date and mode of onset are difficult to evaluate.(ABSTRACT TRUNCATED AT 250 WORDS)