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INTRODUCTION: Life-threatening dermatoses in newborns are versatile and extremely difficult for verification. Infectious diseases, developmental defects and complicated genodermatoses are among the most important causes. MATERIALS AND METHODS: Perinatal history, mother's general state and epidemiologic data are the most important factors for differentiation and proper investigation. Clinical presentation and laboratory findings further support the proper management selection. RESULTS: Treatment options always depend on proper diagnostic verification. CONCLUSIONS: Herein, a thorough review of the most common life-threatening dermatoses in newborns is presented. A diagnostic algorithm and therapeutic clues are provided.