Anaplastic large cell lymphoma (ALCL) is a high-grade T-cell lymphoma. It comprises two systemic subtypes that more frequently affect the lymph nodes and are differentiated based on the presence of ALK gene translocation. Primary cutaneous ALCL and breast implant-associated ALCL should be considered different entities. ALK-positive ALCL is more commonly diagnosed in the first three decades of life, with a slight male predominance and demonstrates a more favorable clinical course and prognosis than its ALK-negative counterpart, which lacks ALK translocation and instead, demonstrates other genetic alterations. Adult patients aged 40–60 years are the most affected individuals by ALK-negative ALCL with a slight male predominance. ALCL manifesting in the oral cavity is very unusual, and tumors usually present as rapidly growing, painful, ulcerated swellings. Patients’ age ranges from 12 to 77 years, depending on the presence of ALK translocation, with a predilection for females. The gingiva/alveolar ridge is the most commonly affected site, followed by the palate. Microscopically, ALCL is characterized by the presence of large pleomorphic cells that stain positively for CD30. Overall survival of patients affected by ALK-positive ALCL may achieve 80%, whereas ALK-negative ALCL is associated with a mean survival ranging from 30% to 40%.