The case of a girl with severe, predominantly mesomelic, intrauterine dwarfism with acromicria is reported. Other anomalies included ligamentary hyperlaxity, clinodactyly of the fifth fingers, and narrow dental arches. Roentgenograms failed to disclose any metaphyseal or epiphyseal anomalies; long bones were narrow and short with thick cortices and the pelvis had an unusual appearance. This case is reminiscent of a constitutional disease with elective involvement of the cortices but distinctive features include the very early onset and the severity of statural growth failure.