Nodular regenerative hyperplasia of the liver, characterised by regenerative nodules distributed throughout the liver in the absence of fibrosis, is a rare but important complication of systemic lupus erythematosus. The main consequence of nodular regenerative hyperplasia of the liver is non-cirrhotic portal hypertension. This condition is probably underdiagnosed, as many of these patients may remain asymptomatic. Furthermore, nodular regenerative hyperplasia of the liver may be misdiagnosed as cirrhosis. We describe three female patients with nodular regenerative hyperplasia of the liver associated with systemic lupus erythematosus. All three patients have clinical manifestations of portal hypertension, and all were initially misdiagnosed as having cryptogenic cirrhosis.