A bizarre disorder was recently encountered in a female child. There was severe lipodystrophy affecting the entire body, generalized muscular hypertrophy, large hands and feet, a markedly protuberant abdomen, hepatomegaly with biochemical and histological evidence of liver dysfunction, a large clitoris, enlarged cystic ovaries and a low fasting blood sugar concentration. This entity is not unique; patients with identical or similar features have previously been described under a variety of titles including the beguiling one of'leprechaunism'(Ziegler, 1928; Hall, Sunderman and Gittings, 1936; Hansen and McQuarrie, 1940; Lawrence, 1946; Corner, 1952; Murray, 1952; Donohue and Uchida, 1954; Tizard, 1954; Berardi-nelli, 1954; Fontan, Verger, Couteau and Pery, 1956; Schwartz, Schafer and Renold, 1960; Seip, 1959)(see Table).