The distinctive combination of clinical and pathological features associated with classic Hodgkin lymphoma (CHL) helped make it the first lymphoma recognized as a discrete entity.1 Unlike the vast majority of mature lymphoid neoplasms, the tendency for CHL to afflict those in their youthful primes, particularly adolescents and young adults (AYA), is a key trait that has been firmly established through epidemiologic investigations. Hodgkin lymphoma is rare in children younger than 5 years, but represents one of the most common cancers diagnosed during the second decade of life (Figure 1) and is the single most commonly diagnosed cancer among adolescents aged 15 to 19 years.2 The International Agency for Research on Cancer (IARC)’s GLOBOCAN 2020 database estimates that there were roughly 83,000 new cases of Hodgkin lymphoma diagnosed worldwide in the year 2020, with roughly one-quarter of those diagnoses occurring in individuals younger than 25 years.3 Cure rates are high using modern regimens;4 however, the individual and societal costs of CHL remain significant due to a variety of late sequelae stemming from the toxicity of initial therapy.5 Knowledge of the biology and molecular underpinnings of this disease has increased substantially in recent years, improving risk stratification,6 inspiring the use of novel therapeutic agents,7 and helping young patients and their families to better understand the genesis of their cancer. With this in mind, the primary aim of this article is to review the salient features of CHL along with known risk factors and lymphomagenic mechanisms, with an emphasis on those most applicable to pediatric and AYA disease.