The most common pathologies of the respiratory organs caused by harmful production factors include dust diseases, bronchial asthma, various variants of toxic lesions and hypersensitive pneumonitis. However, there are also more rare diseases, the awareness of doctors about which is insufficient. These include, in addition to alveolar proteinosis, rare forms of interstitial pneumonia, including lipoid pneumonia. Lipoid pneumonia is a rare lung disease characterized by the accumulation of lipid substances in the alveoli. Cases of development of lipoid pneumonia as a result of inhalation or aspiration of various substances, including oil products are described. The presented clinical observation presents a case of morphologically confirmed disease development in an employee of the petrochemical laboratory who has been in contact with oil vapors and products of its processing for a long time. The main approaches to the treatment of such patients are mentioned. Due to the fact that this pathology is not in the list of occupational diseases approved in the Russian Federation, it was not possible to link the patient’s disease with the profession. The article discusses the features of the legal framework in the field of occupational diseases in the Russian Federation and in the world, proposed harmonization of the list of occupational diseases with the list of the international labour organization as one of the main tasks to improve the legal framework in the field of occupational pathology in Russia. © 2019, Institut meditsiny truda RAMN. All rights reserved.