This is a report of isolated congenital hypoplastic bicuspid pulmonary valve in a 27-year-old white male who had a long history of seizure disorder. Autopsy revealed extensive fibrotic pericarditis, hypoplastic bicuspid, pulmonary valve, and poststenotic dilatation of the pulmonary trunk. The pulmonary valve consisted of sparsely cellular collagenous tissue with minimal to absent elastic elements without evidence of active inflammation.