Two cases of nodular regenerative hyperplasia of the liver coexisting with pulmonary hypertension are described. Regarding its low frequency, nodular regenerative hyperplasia has been found more often than expected in our series of coexisting porto-pulmonary hypertension (2 out of 14 cases, 14 percent). In our 2 cases, pulmonary symptoms occurred before or simultaneously with hepatic symptoms. The high prevalence of this association and the unusual onset setting of symptoms suggest that this association is unfortuitous. A common pathogenesis for pulmonary and hepatic lesions is discussed.