The authors report two cases of marked acro-osteolysis. One patient, a 16-year-old boy, had hypertrophic pulmonary osteoarthropathy associated with bronchiectasis, clubbing of the digits, synovitis, and osseous changes. The other patient, a 55-year-old man, had pachydermoperiostosis as well as synovial hypertrophy, clubbing of the digits, blepharitis, and a cataract. While the association of acro-osteolysis and pachydermoperiostosis has been reported before, this appears to be the first verified case of acro-osteolysis combined with hypertrophic pulmonary osteoarthropathy.