Background: The diagnosis of solitary cutaneous mastocytoma is mainly clinical, based on lesion morphology, the presence of a positive Darier sign, and the absence of systemic involve-ment. Knowledge of this condition is important so that an accurate diagnosis can be made.Objective: To familiarize physicians with the clinical manifestations, diagnosis, evaluation, and man-agement of a solitary cutaneous mastocytoma.Methods: A PubMed search was completed in Clinical Queries using the key term “solitary cutane-ous mastocytoma”. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. Only papers published in English language were included. The information retrieved from the above search was used in the compilation of the present article.Results: Typically, a solitary cutaneous mastocytoma presents as an indurated, erythematous, yellow-brown or reddish-brown macule, papule, plaque or nodule, usually measuring up to 5 cm in diameter. The lesion often has a peau d'orange appearance and a leathery or rubbery consistency. A solitary cu-taneous mastocytoma may urticate spontaneously or when stroked or rubbed (Darier sign). Organo-megaly and lymphadenopathy are characteristically absent. The majority of patients with skin lesions that erupt within the first two years of life have spontaneous resolution of the lesions before puberty. Treatment is mainly symptomatic. Reassurance and avoidance of triggering factors suffice in most cases.Conclusion: The diagnosis is mainly clinical, based on the morphology of the lesion, the presence of a positive Darier sign, and the absence of systemic involvement. A skin biopsy is usually not neces-sary unless the diagnosis is in doubt.