We describe a novel genetic disorder with an unusual multi-organ phenotype of interstitial lung disease with cholesterol pneumonitis, intracranial aneurysms, cerebral calcifications, hypotonia, and liver cirrhosis caused by bi-allelic mutations in FARSB. Other less consistent features include renal disease, intestinal malrotation, and dysmorphic facial features. The gene discussed is FARSB; the disease is interstitial lung disease.