Background: Pulmonary alveolar microlithiasis (PAM) is a rare but not uncommon disease; it showed worldwide distribution either familial or sporadic, the most reported cases in Europe, especially in Turkey. The presence of round shaped little bodies containing concentric calcareous lamellas in pulmonary alveolus is the hallmark of the disease. With this study, we report a case of PAM in Arabic descent, a Saudi child, who represented the most characteristics of the disease in dissociation between definite radiological pattern of the lungs and relatively poor clinical symptoms. Case Presentation: A-9-year-old Arabic female, presented with occasional nonproductive cough for one year, the patient suspected milliary TB and received antituberculous therapy. Examination: Revealed well pleasant child with finger clubbing and clear chest. The patient followed for two years, with marginal deterioration in her general condition. Conclusion: Here, we reported a sporadic case of PAM in Saudi child that presented with nonspecific clinical picture, which resulted in misdiagnosis and consequently improper management. The case proves the slowly progression of the disease.