Three cases of adrenoleukodystrophy are presented with sequential findings by computed tomography (CT). In two cases, CT confirmed not only the findings of several previous reports of a caudocranial direction of low-density white matter and contrast enhancement at the periphery of affected regions during the active demyelinating process, but also two other unusual features: (1) calcification in the affected white matter along the trigones of the lateral ventricles and (2) development of a mass effect during the active demyelinating period. Calcification alone without low-density white matter was the initial CT presentation in one case. Periventricular calcification may be specific for adrenoleukodystrophy in the appropriate clinical setting.