Background : Pulmonary alveolar microlithiasis is a rare condition characterized by a diffuse bilateral filling of the majority of alveoli by calcific concretions called calcospherits. The etiology and pathogenesis are obscure. Osteopetrosis is a heterogenous group of heritable conditions with defect in bone resorption by osteoclasts. Materials & Methods: We report a case of pulmonary alveolar microlithiasis associated with osteopetrosis, which was diagnosed incidentally by high density bones and generalized osteosclerosis in chest x-ray. Results: Association of these two diseases has not been reported before and because of their unknown etiology and presence of hypercalcemia as a probable etiologic factor or predisposing factor and relationship with genetic factors, we report the case.