Background <p>Burkitt’s lymphoma is a highly aggressive B cell non-Hodgkin lymphoma that typically presents with abdominal tumors or jaw involvement, depending on the variant. Involvement of the skull base or paravertebral regions and associated symptoms such as hearing loss are exceedingly rare in Burkitt’s lymphoma. This case highlights the diagnostic challenges when Burkitt’s lymphoma presents with such uncommon manifestations in an adult patient.</p> Case presentation <p>We report a case of a 36-year-old South Asian male individual who presented with generalized body pain, fever, headache, and epigastric discomfort, later developing right-sided facial palsy and hearing loss. Examination revealed mild icterus, lymphadenopathy, and signs of cranial nerve VII involvement. Imaging showed an ill-defined mass at the skull base extending into paravertebral spaces. A comprehensive workup found hematological abnormalities (mild anemia, severe thrombocytopenia) and evidence of hepatic dysfunction. Serologies were notable for positive Epstein–Barr virus IgG. Biopsy of the lesion confirmed Burkitt’s lymphoma, demonstrating the characteristic “starry sky” pattern and a near-100% Ki-67 proliferation index. Immunophenotyping was positive for CD20 and leukocyte common antigen, consistent with high-grade B cell lymphoma. Despite initiation of diagnostic procedures, the patient’s condition deteriorated rapidly. He opted to return to his home country and unfortunately passed away 2 weeks after diagnosis, before definitive therapy could be administered.</p> Conclusion <p>This case underscores the importance of recognizing atypical presentations of Burkitt’s lymphoma, such as skull base involvement and hearing loss. A high index of suspicion and a multimodal diagnostic approach (imaging and histopathology) were crucial for accurate diagnosis. Early identification of such rare presentations is vital to initiate prompt, appropriate therapy and improve patient outcomes.</p>