Introduction: Extranodal non-Hodgkin lymphoma (NHL) of the head and neck represents an uncommon yet clinically significant group of lymphoid malignancies arising from B-cell or T-cell lineages. These tumors are characterized by malignant monoclonal lymphoid proliferation involving extranodal tissues and frequently exhibit aggressive clinical behavior, necessitating prompt diagnosis and treatment. Because of their diverse clinical presentations and rapid progression, they may mimic other benign or malignant conditions, posing diagnostic challenges. Objective: To describe the epidemiological, clinical, radiological, and histopathological characteristics of patients diagnosed with extranodal non-Hodgkin lymphoma of the head and neck and managed at a tertiary referral center. Patients and Methods: A retrospective descriptive study was conducted including 55 patients diagnosed with extranodal head and neck NHL between January 2025 and March 2026 in the Department of Otorhinolaryngology–Head and Neck Surgery at Hôpital des Spécialités, Ibn Sina University Hospital, Rabat, Morocco. Clinical records were reviewed for demographic data, presenting symptoms, endoscopic findings, imaging studies, histopathological and immunophenotypic diagnoses, and anatomical sites of involvement. Results: Fifty-five patients were included, with ages ranging from 3 to 91 years with the mean age was 52.8 ± 23.3 years (mean ± SD). There was a slight male predominance, comprising 31 males (56.4%) and 24 females (43.6%) corresponding to a male-to-female ratio of 1.29:1. The most frequent extranodal sites were the palatine tonsils (25.5%, n = 14), nasopharynx (14.5%, n = 8), oral cavity—including the palate and buccal mucosa—(10.9%, n = 6), facial bones (frontal bone, maxilla, mandible, and nasal pyramid) (10.9%, n = 6), base of tongue (9.1%, n = 5), parapharyngeal space (5.5%, n = 3), external ear and external auditory canal (5.5%, n = 3), parotid gland (5.5%, n = 3), nasal cavity (3.6%, n = 2), thyroid gland (3.6%, n = 2), pharyngolaryngeal region (1.8%, n = 1), frontoethmoidal sinus (1.8%, n = 1), and sternocleidomastoid muscle (1.8%, n = 1). Clinical manifestations varied according to the site of involvement and frequently mimicked other pathological entities. Based on the World Health Organization classification, B-cell lymphomas predominated, accounting for 49 cases (89.1%), whereas T-cell lymphomas represented 6 cases (10.9%). Following diagnosis, all patients were promptly referred to the hematology department for systemic management. Conclusion: Extranodal non-Hodgkin lymphoma of the head and neck is a rare and potentially aggressive malignancy with heterogeneous clinical presentations and a broad spectrum of anatomical involvement. In this series, B-cell lymphomas constituted the predominant histological subtype. Early recognition, timely histopathological diagnosis, and multidisciplinary management are essential to facilitate appropriate treatment and improve patient outcomes