Drug-induced neutropenia and agranulocytosis are serious and potentially life-threatening complications of pharmacotherapy. The aim of the review was to systematise and analyse scientific literature on the prevalence, risk factors, pathogenesis, clinical picture, methods of prevention and treatment of drug-induced neutropenia and agranulocytosis, and medicinal products that most often lead to their development. The analysis revealed that one of the major reasons of drug-induced neutropenia and agranulocytosis is the use of cytotoxic drugs (alkylating agents, antimetabolites, taxanes), non-steroidal anti-inflammatory drugs, antibiotics, antiplatelet, antithyroid, antirheumatic, antiarrhythmic, and antipsychotic drugs. The incidence of drug-induced neutropenia and agranulocytosis is generally low—from 3 to 16 cases per 1 million patients annually. The main risk factors for the development of these conditions include old age, female sex, history of chemotherapy or radiation therapy, malnutrition, and comorbidities. The symptoms in patients with neutropenia and agranulocytosis regardless of etiology can include fever, chills, apathy, myalgia, weakness, pharyngitis, gingivitis, sinusitis, stomatitis, bronchitis, and sepsis. There are no practical methods for early diagnosis or prevention of drug-induced neutropenia and agranulocytosis. Successful management of these conditions relies on timely identification and immediate withdrawal of the potential causative drug. Prevention of chemotherapy-associated neutropenia may consist in reducing the dose of the chemotherapeutic agent. Practicing physicians should be aware of the risk of drug-induced neutropenia and agranulocytosis. It is important to raise awareness among healthcare professionals of the methods of diagnosis and prevention of these conditions, as well as specific aspects of managing patients with these conditions.