Urbach — Wiethe disease (lipoid proteinosis) is a rare genetic dermatosis caused by mutation in ECM1 gene on chromosome 1, that encodes extracellular matrix protein 1. This disease can affect the skin, upper respiratory tract, brain, less often — other visceral organs. There are 400 cases of this disease described in the literature, among which 30 are registered in India. We present a description of our observation of 9-years-old girl with Urbach — Wiethe disease. Clinical symptoms in the form of hoarseness and skin rashes occurred in patient at an early childhood, but she was observed by a doctor in her home area with «atopic dermatitis» diagnosis. Anamnesis and clinical pattern data, as well as results of performed examination (histological skin examination, MRI/CT of the brain) allowed to confirm lipoid proteinosis diagnosis. Whereas this pathology is rare in clinical practice, raising awareness of dermatovenerologists, otorhinolaryngologists, neurologists and doctors of other specialties will allow to timely diagnose this disease. Patients with lipoid proteinosis should be provided with genetic counselling to identify ECM1 mutation variants and to inform patients and their relatives about the nature, inheritance ways and disease consequences as well as potential risks for offspring.