Montelukast the leukotriene receptor antagonist is an anti-inflammatory drug that causes bronchodilation and for this reason it is used to improve inflammatory states in asthma and allergic rhinitis. Montelukast is generally considered a safe drug with the occurrence of a few adverse drug reactions (ADRs) and anti-leucotrienes are usually well-tolerated by adults and young patients. Starting from these premises the purpose of this review is so give un up-to-date scenario about skin adverse reactions due to Montelukast administration. Only few cases were reported during last years, however interestingly some recent reports let us enlarging our ADR data about Montelukast. We decided to divide the paragraph into sections evaluating the following skin lesions: vasculitic lesions, rash, urticaria and angioedema. As described in the results, CSS were the most frequent cases reported, belonging to the Vasculitis category. We speculated several mechanisms leading to the spread of the skin reactions. Montelukast still remains a safe drug used for the treatment of severe and moderate asthma. However, for some reasons still in course of analysis, in rare cases patients could develop ADR. Among these, about half of the patients show skin signs as rash, vescicles, bullous skin, purpura, maculopapular cutis, erythematous exanthema, urticaria and angioedema. Most of these symptoms are a consequence of the onset of a vasculitis as CSS and allergic granulomatous angiitis. In many cases the onset of the reactions happen within the first months of intake. For this reason, the prescribing physicians should be alert for signs, symptoms and genetic predisposition of these skin diseases.