Introduction:

Van der Knaap Disease or Megalencepahalic Leukoencephalopathy is a rare disorder of white matter of brain. It is common in Agarwal families in India. We present a case of Van der Knaap disease from India that is not from Agarwal community.

Case: 10 yr boy presented with history of regression of milestones and increasing head size. MRI revealed cysts in temporal and frontal lobes. Very slow progression of disease and classical MRI findings we diagnosed this case as van der Knaap disease.

Conclusion: van der Knaap disease though common in Agarwal community can occur in other communities from India.