Since its first description in 1951 by Mantz and Craig, pulmonary hypertension associated with portal hypertension has been observed more frequently. In a recent prospective study Hadengue et al. reported 2% incidence of pulmonary hypertension in patients with portal hypertension. Thus this simultaneous occurrence can no longer be considered to be coincidental. The etiology remains unclear. It is most likely that vasoactive substances (,)normally metabolized by the liver(,) may gain have gained access to the pulmonary circulation through portosystemic collaterals in portal hypertension. In genetically susceptible individuals, these substances could lead to pulmonary hypertension by inducing vasoconstriction or direct toxic damage to the wall of the small pulmonary arteries. A recent case of pulmonary hypertension in a 49-year-old woman with portal hypertension due to liver cirrhosis is reported as well as a review of the literature.