Intracranial tuberculoma represents one of the most severe complications of central nervous system tuberculosis (TB), with an incidence that is relatively low. In cases of intracranial tuberculoma, patients may develop drug toxicity and/or immune reconstitution inflammatory syndrome (IRIS) while receiving anti-TB treatment. The current study presented the case of a seven-year-old female patient with intracranial tuberculoma who developed drug-induced hepatotoxicity and IRIS during the course of treatment. During the follow-up of the patient, anti-TB drug-induced hepatitis developed, which led to the discontinuation of the drug twice. In the seventh month of treatment, cranial MRI showed the progression of tuberculoma lesions. The possibility of IRIS or treatment failure was considered and the treatment was restarted with steroids and non-hepatotoxic anti-TB drugs. With steroid and anti-TB treatment, the lesions regressed almost completely and the neurological deficit regressed. Patients receiving treatment should be followed up closely due to the possible side effects of anti-TB drugs, especially IRIS, which develops as an immune restructuring response during the recovery of the immune system.