Atypical mycobacteriosis is a rare cause of cervical lymphadenitis that most frequently affects immunoincompetent children between the ages of 12 months and 5 years. The typical clinical manifestation is a painless unilateral cervical mass. The nonspecific clinical symptoms and laboratory parameters complicate diagnosis and, therefore, therapeutic management. Various therapeutic options, including surgery, antimycobacterial drug therapy and wait-and-scan approaches are discussed in the literature. Complete surgical excision has become the established treatment of choice. However, controlled randomized studies that clearly demonstrate the benefits of a particular type of therapy are lacking.