* Consultant thoracic surgeon, Erbil, Iraq. ** Kurdistan board for medical specialties, Erbil, Iraq. *** Department of surgery, college of medicine, Hawler medical university, Erbil, Iraq. Introduction Pulmonary alveolar microlithiasis is uncommon chronic lung disease characterized by the presence of calcific concretions within the alveolar spaces. Pulmonary alveolar microlithiasis was first described by Friedrich in 1856 and then by Harbitz in 1918. O’Neill reported a family with pulmonary alveolar microlithiasis in 1967 and noted that the total number of recorded cases at that time was seventy. It occurs sporadically and the symptoms of lung disorder with restrictive pattern start in most cases in the third and fourth decades of life. Pulmonary alveolar microlithiasis is regarded as an autosomal recessive lung disease with apparently no important gender predominance. As of 2004, 576 cases have been studied, and most have originated from Europe (42.7%) and Asia (40.6%). The major finding on chest radiograph is sand like diffuse symmetric micronodular calcification which is predominant in the middle and lower lobes with sparing of apices. We report this case because it is very rare. We aim to emphasize a high index of suspicion for this uncommon disorder to avoid misdiagnosis of miliary tuberculosis and sarcoidosis and as far as we are aware this is the first case to be reported in Erbil, Kurdistan, Iraq. Case presentation: 39-year-old housewife, presented with progressive shortness of breath and cough for the last 10 years. She had no history of fever or weight loss, nor history of dust inhalation or smoking. No history of similar condition in the family was noted. For the last two years, the case has been treated elsewhere as miliary tuberculosis based on chest x-ray findings, but with no clinical or radiological response. Physical examination revealed crackles at auscultation of the chest. Cardiac auscultation was normal and no cyanosis or peripheral edema was observed. Sputum examination for acid-fast bacilli was not performed and the routine laboratory findings were unremarkable including serum calcium and phosphorus. Pulmonary function test showed mild restrictive ventilator defect (mildly increased FEV1/FVC ratio). Arterial blood gas analysis showed reduction in O2saturation of 85%, and echocardiography showed no significant abnormal findings. Serial chest x-ray demonstrated diffuse bilateral dense micro nodular infiltrate predominantly at mid and lower zones with obliteration of cardiac and diaphragmatic borders (Figure 1). High-resolution computed tomography revealed diffuse minute pulmonary calcification, distributed predominantly over basal and posterior Background and objective: Pulmonary alveolar microlithiasis is rare disease characterized by the presence of numerous small calculi within the alveolar spaces. Case report: 39-year-old female with pulmonary alveolar microlithiasis is described, in which the diagnosis was suspected clinically and radiologically and confirmed histopathologically. The clinical, pathogenesis and pathology are briefly reviewed.