Background:Type 1 neurofibromatosis is a common neurocutaneous disease characterised by café-au-lait macules, neurofibromas, plexiform neurofibromas, axillary freckling, optic glioma, and Lisch nodules.Main observations:We report a 10-year-old boy with a rare presentation of type 1 neurofibromatosis, presenting with bilateral, multiple linear asymptomatic nodules extending from the axillae to the wrists, bilateral thickening of ulnar and supraorbital nerves, and 16 cafe café-au-lait macules over 0.5 cm in diameter on trunk and extremities, and bilateral axillary freckles. We diagnosed neurofibromatosis 1 clinically on the basis of NIH diagnostic criteria.Conclusion:As the clinical criteria were fulfilled, we believe that excisional biopsy as an invasive procedure was not clinically indicated for a 10-year-old child in this scenario. The patient has to be followed-up for possible malignant transformation of tumors.